Materiały źródłowe

• #1 POEMS syndrome – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/poems-syndrome/symptoms-causes/syc-20352678

  POEMS syndrome is a rare blood disorder that damages nerves and affects other parts of the body. Symptoms may include weak legs, trouble breathing, a larger spleen or liver, and skin changes. […] Without treatment, POEMS syndrome gets worse quickly. It can become life-threatening. So early diagnosis is vital. […] The symptoms of POEMS syndrome make up its name. Symptoms most often appear over weeks to months. They include the following: Polyneuropathy. This may include numbness, tingling and weakness in the legs. In time, it might affect the hands. It also involves trouble breathing. Polyneuropathy must be present for a POEMS diagnosis. Organomegaly. This is an enlarged spleen, liver or lymph nodes. Endocrinopathy and edema. Atypical hormone levels can cause an underactive thyroid, also called hypothyroidism. The hormone levels also can cause diabetes, sexual issues, tiredness, and swelling in arms and legs. Monoclonal protein. This is a protein that atypical plasma cells in bone marrow make. The monoclonal protein goes into the bloodstream. This must be present for the diagnosis of POEMS syndrome. Monoclonal protein often is linked with bones getting harder or thicker. Skin changes. This may involve having more color than usual on the skin. There may be red spots, which may be harder to see on Black or brown skin. The skin might be thicker. There might be more hair on the face or the legs.

• #2 POEMS Syndrome: What It Is, Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/17985-poems-syndrome

  POEMS syndrome is a rare blood disorder that can impact multiple body systems. With POEMS, your body makes abnormal plasma cells that multiply out of control. These cells release a substance called a monoclonal protein (or M-protein) into your blood. Having too many abnormal plasma cells and too much M-protein can damage your nerves and organs. […] The most common signs of POEMS syndrome are polyneuropathy, organomegaly, endocrinopathy, having too many M-proteins and skin changes. Everyone with POEMS has polyneuropathy and too many M-proteins. Other signs and symptoms vary. […] Symptoms of polyneuropathy are the most common sign of POEMS syndrome. Often, people notice pain thats most intense in their legs and feet, but their arms and hands may also be affected. Polyneuropathy may also feel like: Weakness. Tingling. Numbness. Prickling. Burning. A pins-and-needles sensation.

• #3 Comprehensive Diagnosis and Management of POEMS Syndrome

  https://pmc.ncbi.nlm.nih.gov/articles/PMC9624442/

  Polyneuropathy Organomegaly, Endocrinopathy, Monoclonal protein and Skin changes syndrome is a rare multisystem condition with a range of manifestations which are often overlooked as trivial comorbidities, until their whole triggers the possibility of the diagnosis. The diagnosis is typically delayed by 1216 months, by which time patients can be severely disabled. […] Patients present most often to neurologists or hematologists with progressive peripheral neuropathy and evidence a of monoclonal plasma cell disorder. There are a range of systemic manifestations which are noted but often overlooked as trivial comorbidities. The diagnosis is typically delayed by 1216 months, by which time patients can be severely disabled and bed or wheelchair-bound with established neuropathy. […] Treatment, in the main, improves functionality and leads to encouraging rates of progression-free survival. Furthermore dramatic improvements in overall survival have been achieved in the last 20 years.

• #4 POEMS syndrome – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/poems-syndrome/symptoms-causes/syc-20352678

  POEMS syndrome is a rare blood disorder that damages nerves and affects other parts of the body. Symptoms may include weak legs, trouble breathing, a larger spleen or liver, and skin changes. […] Without treatment, POEMS syndrome gets worse quickly. It can become life-threatening. So early diagnosis is vital. […] The symptoms of POEMS syndrome make up its name. Symptoms most often appear over weeks to months. They include the following: Polyneuropathy. This may include numbness, tingling and weakness in the legs. In time, it might affect the hands. It also involves trouble breathing. Polyneuropathy must be present for a POEMS diagnosis. Organomegaly. This is an enlarged spleen, liver or lymph nodes. Endocrinopathy and edema. Atypical hormone levels can cause an underactive thyroid, also called hypothyroidism. The hormone levels also can cause diabetes, sexual issues, tiredness, and swelling in arms and legs. Monoclonal protein. This is a protein that atypical plasma cells in bone marrow make. The monoclonal protein goes into the bloodstream. This must be present for the diagnosis of POEMS syndrome. Monoclonal protein often is linked with bones getting harder or thicker. Skin changes. This may involve having more color than usual on the skin. There may be red spots, which may be harder to see on Black or brown skin. The skin might be thicker. There might be more hair on the face or the legs.

• #5 POEMS syndrome – Autoimmune Association

  https://autoimmune.org/disease-information/poems-syndrome/

  POEMS syndrome can be misdiagnosed because the signs and symptoms mimic those of other disorders. POEMS syndrome progresses rapidly and may become life-threatening, so early diagnosis is important. […] Signs and symptoms may include progressive sensorimotor polyneuropathy; enlarged liver, spleen, and/or lymph nodes; a disorder of the endocrine glands (often with multiple abnormalities); a monoclonal plasma cell proliferative disorder; and darkening of the skin (hyperpigmentation). Various other symptoms may also occur and may vary among affected people. POEMS syndrome is a chronic disorder, with a median survival time of 8-14 years.

• #6 POEMS syndrome – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/poems-syndrome/symptoms-causes/syc-20352678

  POEMS syndrome is a rare blood disorder that damages nerves and affects other parts of the body. Symptoms may include weak legs, trouble breathing, a larger spleen or liver, and skin changes. […] Without treatment, POEMS syndrome gets worse quickly. It can become life-threatening. So early diagnosis is vital. […] The symptoms of POEMS syndrome make up its name. Symptoms most often appear over weeks to months. They include the following: Polyneuropathy. This may include numbness, tingling and weakness in the legs. In time, it might affect the hands. It also involves trouble breathing. Polyneuropathy must be present for a POEMS diagnosis. Organomegaly. This is an enlarged spleen, liver or lymph nodes. Endocrinopathy and edema. Atypical hormone levels can cause an underactive thyroid, also called hypothyroidism. The hormone levels also can cause diabetes, sexual issues, tiredness, and swelling in arms and legs. Monoclonal protein. This is a protein that atypical plasma cells in bone marrow make. The monoclonal protein goes into the bloodstream. This must be present for the diagnosis of POEMS syndrome. Monoclonal protein often is linked with bones getting harder or thicker. Skin changes. This may involve having more color than usual on the skin. There may be red spots, which may be harder to see on Black or brown skin. The skin might be thicker. There might be more hair on the face or the legs.

• #7 POEMS Syndrome: 2019 Update on diagnosis, risk-stratification, and management – PubMed

  https://pubmed.ncbi.nlm.nih.gov/31012139/

  Polyneuropathy, organomegaly, endocrinopathy, M-protein, skin changes (POEMS) syndrome is a paraneoplastic syndrome due to an underlying plasma cell neoplasm. The major criteria for the syndrome are polyradiculoneuropathy, clonal plasma cell disorder (PCD), sclerotic bone lesions, elevated vascular endothelial growth factor, and the presence of Castleman disease. Minor features include organomegaly, endocrinopathy, characteristic skin changes, papilledema, extravascular volume overload, and thrombocytosis. […] The diagnosis of POEMS syndrome is made with three of the major criteria, two of which must include polyradiculoneuropathy and clonal PCD, and at least one of the minor criteria. […] Risk factors include low serum albumin, age, pleural effusion, pulmonary hypertension, and reduced eGFR.

• #8 POEMS Syndrome: What It Is, Symptoms, Causes, and Treatment

  https://www.verywellhealth.com/poems-syndrome-7558580

  Symptoms can vary, potentially involving abdominal swelling, shortness of breath, and pain. […] The diverse symptoms of POEMS syndrome can develop at any time during the disease course, and they dont have to follow a specific sequence. You might have some symptoms but not others. […] Symptoms of POEMS can include: Swelling in arms and legs: Monoclonal protein accumulation, plasma cell excess, endocrine disorders, or organ failure may cause swelling of the arms and legs. Unexplained weight loss: Organ failure or endocrine disease can affect weight. Bone and joint pain: Hardened areas of the bone can lead to pain and an increased risk of fractures. Vision problems: Papilledema (swelling in the eyes) can cause vision loss. Trouble breathing: Nerve disease can impair the muscles that control breathing. Fatigue: Many of the effects of POEMS syndrome can cause fatigue. A few effects that may contribute to fatigue include thyroid disease, shortness of breath, and swelling. Fever: Sometimes, elevated proteins or plasma cells in the blood can cause a fever.

• #9

  https://link.springer.com/article/10.1007/s00415-018-9110-6

  POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, M-protein, skin changes) is a rare paraneoplastic syndrome, caused by a plasma cell proliferative disorder, which is most commonly lambda restricted. The neurological hallmark, which forms one of the mandatory criteria for diagnosis, is a subacute onset demyelinating neuropathy, which can be rapidly disabling and painful. […] Patients typically describe a subacute, painful, distal neuropathy. If POEMS syndrome is suspected, a thorough systemic examination and timely organisation of relevant investigations are required to elicit all features that might aid diagnosis. […] Patients typically present with a subacute, distal, symmetrical, sensorimotor neuropathy, frequently painful, with allodynia and hyperpathia. Neuropathy is a common first clinical feature, and may be the only feature at first presentation.

• #10 POEMS Syndrome: Background, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/1097031-overview

  POEMS syndrome is a rare multisystemic disease that occurs in the setting of a plasma cell dyscrasia. […] The polyneuropathy associated with POEMS syndrome is bilateral and symmetric. It involves both motor and sensory nerves, begins distally, and has a progressive proximal spread. Associated cranial or autonomic nerves are not involved. Both demyelination and axonal degeneration are noted. […] Multiple endocrinopathies have been associated with POEMS syndrome, and most patients have more than one endocrine abnormality. Many of the abnormalities noted can be explained by elevations in estrogen levels. Impotence and gynecomastia are common among men. Amenorrhea is common among women. Diabetes mellitus and glucose intolerance are also noted in many patients. Other associated endocrinopathies include hypothyroidism, hyperprolactinemia, and hypoparathyroidism.

• #11 POEMS syndrome – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/poems-syndrome/symptoms-causes/syc-20352678

  POEMS syndrome is a rare blood disorder that damages nerves and affects other parts of the body. Symptoms may include weak legs, trouble breathing, a larger spleen or liver, and skin changes. […] Without treatment, POEMS syndrome gets worse quickly. It can become life-threatening. So early diagnosis is vital. […] The symptoms of POEMS syndrome make up its name. Symptoms most often appear over weeks to months. They include the following: Polyneuropathy. This may include numbness, tingling and weakness in the legs. In time, it might affect the hands. It also involves trouble breathing. Polyneuropathy must be present for a POEMS diagnosis. Organomegaly. This is an enlarged spleen, liver or lymph nodes. Endocrinopathy and edema. Atypical hormone levels can cause an underactive thyroid, also called hypothyroidism. The hormone levels also can cause diabetes, sexual issues, tiredness, and swelling in arms and legs. Monoclonal protein. This is a protein that atypical plasma cells in bone marrow make. The monoclonal protein goes into the bloodstream. This must be present for the diagnosis of POEMS syndrome. Monoclonal protein often is linked with bones getting harder or thicker. Skin changes. This may involve having more color than usual on the skin. There may be red spots, which may be harder to see on Black or brown skin. The skin might be thicker. There might be more hair on the face or the legs.

• #12 POEMS Syndrome: What It Is, Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/17985-poems-syndrome

  POEMS syndrome is a rare blood disorder that can impact multiple body systems. With POEMS, your body makes abnormal plasma cells that multiply out of control. These cells release a substance called a monoclonal protein (or M-protein) into your blood. Having too many abnormal plasma cells and too much M-protein can damage your nerves and organs. […] The most common signs of POEMS syndrome are polyneuropathy, organomegaly, endocrinopathy, having too many M-proteins and skin changes. Everyone with POEMS has polyneuropathy and too many M-proteins. Other signs and symptoms vary. […] Symptoms of polyneuropathy are the most common sign of POEMS syndrome. Often, people notice pain thats most intense in their legs and feet, but their arms and hands may also be affected. Polyneuropathy may also feel like: Weakness. Tingling. Numbness. Prickling. Burning. A pins-and-needles sensation.

• #13 Comprehensive Diagnosis and Management of POEMS Syndrome

  https://pmc.ncbi.nlm.nih.gov/articles/PMC9624442/

  Sensorimotor neuropathy is the most common initial symptom of POEMS syndrome. This typically starts distally, in the feet and progresses proximally in a length-dependent fashion; the hands only become affected once neuropathy reaches the knees. Deterioration may occur in days-to-weeks in some and weeks-to-months in others. Patients initially complain of a cramping, tight sensation in the calves initially as if they have run too far and later, bilateral, hyperesthetic and dysaesthetic neuropathic pain. Weakness is striking in its distal selectivity (below the ankles and the intrinsic hand muscles) and differs from chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) in its lack of proximal weakness for the earlier course. Early increased tendency to trip over feet may be due to footdrop.

• #14 POEMS syndrome – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/poems-syndrome/symptoms-causes/syc-20352678

  POEMS syndrome is a rare blood disorder that damages nerves and affects other parts of the body. Symptoms may include weak legs, trouble breathing, a larger spleen or liver, and skin changes. […] Without treatment, POEMS syndrome gets worse quickly. It can become life-threatening. So early diagnosis is vital. […] The symptoms of POEMS syndrome make up its name. Symptoms most often appear over weeks to months. They include the following: Polyneuropathy. This may include numbness, tingling and weakness in the legs. In time, it might affect the hands. It also involves trouble breathing. Polyneuropathy must be present for a POEMS diagnosis. Organomegaly. This is an enlarged spleen, liver or lymph nodes. Endocrinopathy and edema. Atypical hormone levels can cause an underactive thyroid, also called hypothyroidism. The hormone levels also can cause diabetes, sexual issues, tiredness, and swelling in arms and legs. Monoclonal protein. This is a protein that atypical plasma cells in bone marrow make. The monoclonal protein goes into the bloodstream. This must be present for the diagnosis of POEMS syndrome. Monoclonal protein often is linked with bones getting harder or thicker. Skin changes. This may involve having more color than usual on the skin. There may be red spots, which may be harder to see on Black or brown skin. The skin might be thicker. There might be more hair on the face or the legs.

• #15

  https://journals.lww.com/hemasphere/fulltext/2022/11000/comprehensive_diagnosis_and_management_of_poems.12.aspx

  Polyneuropathy Organomegaly, Endocrinopathy, Monoclonal protein and Skin changes (POEMS) syndrome is a rare multisystem condition of undefined pathogenesis. Patients present most often to neurologists or hematologists with progressive peripheral neuropathy and evidence a of monoclonal plasma cell disorder. There are a range of systemic manifestations which are noted but often overlooked as trivial comorbidities. The diagnosis is typically delayed by 1216 months, by which time patients can be severely disabled and bed or wheelchair-bound with established neuropathy. […] Sensorimotor neuropathy is the most common initial symptom of POEMS syndrome. This typically starts distally, in the feet and progresses proximally in a length-dependent fashion; the hands only become affected once neuropathy reaches the knees. Deterioration may occur in days-to-weeks in some and weeks-to-months in others. Patients initially complain of a cramping, tight sensation in the calves initially as if they have run too far and later, bilateral, hyperesthetic and dysaesthetic neuropathic pain. Weakness is striking in its distal selectivity (below the ankles and the intrinsic hand muscles) and differs from chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) in its lack of proximal weakness for the earlier course. Early increased tendency to trip over feet may be due to footdrop. The distal, ataxic sensory neuropathy associated with IgM anti-MAG antibodies is entirely different. Indeed, the clinical neuropathy of POEMS is almost entirely clinically discernible from all other paraprotein-associated neuropathies by its clinical and electrophysiological features.

• #16 Comprehensive Diagnosis and Management of POEMS Syndrome

  https://pmc.ncbi.nlm.nih.gov/articles/PMC9624442/

  Sensorimotor neuropathy is the most common initial symptom of POEMS syndrome. This typically starts distally, in the feet and progresses proximally in a length-dependent fashion; the hands only become affected once neuropathy reaches the knees. Deterioration may occur in days-to-weeks in some and weeks-to-months in others. Patients initially complain of a cramping, tight sensation in the calves initially as if they have run too far and later, bilateral, hyperesthetic and dysaesthetic neuropathic pain. Weakness is striking in its distal selectivity (below the ankles and the intrinsic hand muscles) and differs from chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) in its lack of proximal weakness for the earlier course. Early increased tendency to trip over feet may be due to footdrop.

• #17 POEMS Syndrome: Background, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/1097031-overview

  The prognosis depends on the extent of the underlying plasma cell disorder and its response to treatment. The prognosis is best for patients with a single lytic lesion, worst for those with a plasma cell disorder involving the bone marrow, and intermediate for those with multiple lytic bone lesions. When the plasma cell disorder responds to treatment, all other symptoms usually improve or resolve completely. […] The natural course of POEMS syndrome is chronic, with a reported median survival of approximately a decade (8-13.8 y). […] Central and peripheral nervous system involvement can lead to significant morbidity and mortality. […] The neurologic sequelae of POEMS syndrome cause approximately 50% of patients with POEMS syndrome to become bedridden.

• #18 POEMS syndrome

  https://dermnetnz.org/topics/poems-syndrome

  Hypogonadism is the most common abnormality. […] Darkened skin colour (hyperpigmentation) has been seen in about 50-90% patients with POEMS syndrome. […] POEMS syndrome may also cause weight loss and fatigue. […] POEMS syndrome is a chronic disease. The median survival is around a decade (8-14 years), which is 3 times longer than patients with multiple myeloma. However, many patients are bedridden due to neuropathy (50%). […] Shorter survival is associated with nail clubbing and extravascular fluid overload.

• #19 POEMS syndrome

  https://dermnetnz.org/topics/poems-syndrome

  A common initial presentation of POEMS syndrome is with progressive weakness and swelling of the peripheries. […] Peripheral neuropathy (disorder of peripheral nerves) is often prominent, causing weakness in the feet and later in the arms. In more than half of patients, the weakness is severe, so that they are unable to climb stairs, get up from a seated position, or grip objects. Patients are often eventually confined to a wheelchair. […] POEMS syndrome can also cause reduced sensation. Pinprick, vibration and sense of position (proprioception) are affected the most, whereas temperature and pain sensation usually stay normal. […] Organomegaly (enlargement of internal organs) is present in up to 50% patients and is generally mild. […] Most patients with POEMS syndrome have endocrinopathy (disorder of endocrine glands), often with multiple abnormalities.

• #20 POEMS Syndrome: What It Is, Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/17985-poems-syndrome

  Imaging procedures may show that you have an enlarged: Liver (hepatomegaly). Spleen (splenomegaly). Lymph nodes (lymphadenopathy). […] Low levels of sex hormones are the most common sign of endocrinopathy with POEMS. […] High levels of M-protein are a sign of POEMS. Too much M-protein can cause tumors to form in your blood. Excessive M-protein can cause your bones to thicken or harden (osteosclerosis). […] A variety of skin changes may take place, including: Darker skin. Thicker skin. White fingernails. Increased hair growth on your face and legs. Growth of blood vessels (particularly on your chest) that look like small cherries. […] Other symptoms may include: Swelling in your arms and legs. Unexplained weight loss. Bone and joint pain. Vision problems. Trouble breathing. Fatigue. Fever.

• #21 POEMS Syndrome: What It Is, Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/17985-poems-syndrome

  Imaging procedures may show that you have an enlarged: Liver (hepatomegaly). Spleen (splenomegaly). Lymph nodes (lymphadenopathy). […] Low levels of sex hormones are the most common sign of endocrinopathy with POEMS. […] High levels of M-protein are a sign of POEMS. Too much M-protein can cause tumors to form in your blood. Excessive M-protein can cause your bones to thicken or harden (osteosclerosis). […] A variety of skin changes may take place, including: Darker skin. Thicker skin. White fingernails. Increased hair growth on your face and legs. Growth of blood vessels (particularly on your chest) that look like small cherries. […] Other symptoms may include: Swelling in your arms and legs. Unexplained weight loss. Bone and joint pain. Vision problems. Trouble breathing. Fatigue. Fever.

• #22 POEMS Syndrome: What It Is, Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/17985-poems-syndrome

  Imaging procedures may show that you have an enlarged: Liver (hepatomegaly). Spleen (splenomegaly). Lymph nodes (lymphadenopathy). […] Low levels of sex hormones are the most common sign of endocrinopathy with POEMS. […] High levels of M-protein are a sign of POEMS. Too much M-protein can cause tumors to form in your blood. Excessive M-protein can cause your bones to thicken or harden (osteosclerosis). […] A variety of skin changes may take place, including: Darker skin. Thicker skin. White fingernails. Increased hair growth on your face and legs. Growth of blood vessels (particularly on your chest) that look like small cherries. […] Other symptoms may include: Swelling in your arms and legs. Unexplained weight loss. Bone and joint pain. Vision problems. Trouble breathing. Fatigue. Fever.

• #23 A Review of POEMS Syndrome

  https://www.cancernetwork.com/view/review-poems-syndrome

  The presence of a monoclonal plasma cell disorder is required to make the diagnosis. […] Organomegaly manifests commonly as hepatomegaly, splenomegaly, and/or lymphadenopathy. […] The endocrine features are diverse and may involve any endocrine gland function, hypothalamic function, or pituitary function. Erectile dysfunction is a common first or second symptom in male patients with POEMS. […] Skin manifestations include hyperpigmentation, a recent outcropping of hemangiomas, hypertrichosis, dependent rubor and acrocyanosis, white nails, sclerodermoid changes, flushing, or nail clubbing. […] Papilledema is present in at least one-third of patients. […] Extravascular overload most commonly manifests as peripheral edema, but pleural effusion, ascites, and pericardial effusions are also common.

• #24 POEMS Syndrome – Treatment abroad

  https://www.gsdinternational.com/conditions/poems-syndrome

  The onset symptoms are mainly associated with bilateral polyneuropathy, which affects both motor and sensory fibers of the lower extremities and manifests itself as muscle weakness in the arms and legs, tingling, numbness, burning pain and a lack of sensitivity. […] Organomegaly, that is, an increase in the size of some internal organs, most often lymph nodes, liver, spleen and, less often, heart, can be found in about half of patients. Among the endocrinological changes, the most common are hypothyroidism and adrenal insufficiency; Also, sexual function is often involved with an increase in the level of estrogen in the blood, which determines impotence and gynecomastia in men, that is, excessive development of the mammary glands, and in women – the absence of menstruation and hypertrichosis. Also common: diabetes / glucose intolerance.

• #25 POEMS syndrome – Wikipedia

  https://en.wikipedia.org/wiki/POEMS_syndrome

  Papilledema, polyneuropathy, edema, hyperpigmentation, hypogonadism, visceromegaly, amenorrhea, gynecomastia, erectile dysfunction, and testicular atrophy. […] The signs and symptoms of POEMS syndrome are highly variable. This often leads to long delays (e.g. 13-18 months) between the onset of initial symptoms and diagnosis. […] POEMS syndrome typically begins in middle age the average age at onset is 50 and affects up to twice as many men as women. […] Subacute, distal, symmetrical sensorimotor neuropathy with allodynia and hyperpathia is the most frequent presentation of POEMS syndrome. Neuropathy is often the first trait, and it may be the only initial symptom. […] Approximately 84% of POEMS syndrome patients have characteristics of several endocrinopathies. Hypogonadism is the most prevalent endocrine disorder, followed by thyroid anomalies, glucose metabolism defects, and adrenal insufficiency.

• #26 Poems Syndrome, Cryoglobulinemia, and Heavy-Chain Disease | Oncohema Key

  https://oncohemakey.com/poems-syndrome-cryoglobulinemia-and-heavy-chain-disease/

  The most notable symptoms include the constellation of neuropathy and any of the following: monoclonal protein (especially λ-light chain), thrombocytosis, anasarca, or papilledema. All the features of the acronym are not required to make the diagnosis. […] The peripheral neuropathy is the dominant characteristic, and it is ascending, symmetrical, and affecting both sensation and motor function. In our experience, pain may be a dominant feature in about 10% to 15% of patients, and in one report as many as 76% of patients had painful neuropathy. […] Depending on the series, 45% to 85% of patients will have any combination of splenomegaly, hepatomegaly, and/or lymphadenopathy. […] Endocrinopathy is a central but poorly understood feature of POEMS. In a recent series, approximately 84% of patients had a recognized endocrinopathy, with hypogonadism as the most common endocrine abnormality, followed by thyroid abnormalities, glucose metabolism abnormalities, and lastly by adrenal insufficiency.

• #27 POEMS syndrome

  https://dermnetnz.org/topics/poems-syndrome

  Hypogonadism is the most common abnormality. […] Darkened skin colour (hyperpigmentation) has been seen in about 50-90% patients with POEMS syndrome. […] POEMS syndrome may also cause weight loss and fatigue. […] POEMS syndrome is a chronic disease. The median survival is around a decade (8-14 years), which is 3 times longer than patients with multiple myeloma. However, many patients are bedridden due to neuropathy (50%). […] Shorter survival is associated with nail clubbing and extravascular fluid overload.

• #28 A Review of POEMS Syndrome

  https://www.cancernetwork.com/view/review-poems-syndrome

  The presence of a monoclonal plasma cell disorder is required to make the diagnosis. […] Organomegaly manifests commonly as hepatomegaly, splenomegaly, and/or lymphadenopathy. […] The endocrine features are diverse and may involve any endocrine gland function, hypothalamic function, or pituitary function. Erectile dysfunction is a common first or second symptom in male patients with POEMS. […] Skin manifestations include hyperpigmentation, a recent outcropping of hemangiomas, hypertrichosis, dependent rubor and acrocyanosis, white nails, sclerodermoid changes, flushing, or nail clubbing. […] Papilledema is present in at least one-third of patients. […] Extravascular overload most commonly manifests as peripheral edema, but pleural effusion, ascites, and pericardial effusions are also common.

• #29 POEMS syndrome – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/poems-syndrome/symptoms-causes/syc-20352678

  POEMS syndrome is a rare blood disorder that damages nerves and affects other parts of the body. Symptoms may include weak legs, trouble breathing, a larger spleen or liver, and skin changes. […] Without treatment, POEMS syndrome gets worse quickly. It can become life-threatening. So early diagnosis is vital. […] The symptoms of POEMS syndrome make up its name. Symptoms most often appear over weeks to months. They include the following: Polyneuropathy. This may include numbness, tingling and weakness in the legs. In time, it might affect the hands. It also involves trouble breathing. Polyneuropathy must be present for a POEMS diagnosis. Organomegaly. This is an enlarged spleen, liver or lymph nodes. Endocrinopathy and edema. Atypical hormone levels can cause an underactive thyroid, also called hypothyroidism. The hormone levels also can cause diabetes, sexual issues, tiredness, and swelling in arms and legs. Monoclonal protein. This is a protein that atypical plasma cells in bone marrow make. The monoclonal protein goes into the bloodstream. This must be present for the diagnosis of POEMS syndrome. Monoclonal protein often is linked with bones getting harder or thicker. Skin changes. This may involve having more color than usual on the skin. There may be red spots, which may be harder to see on Black or brown skin. The skin might be thicker. There might be more hair on the face or the legs.

• #30 POEMS Syndrome – Endocrine and Metabolic Disorders – Merck Manual Professional Edition

  https://www.merckmanuals.com/professional/endocrine-and-metabolic-disorders/polyglandular-deficiency-syndromes/poems-syndrome

  POEMS syndrome is a rare paraneoplastic disorder and is probably caused by circulating immunoglobulins caused by a plasma cell disorder. […] Patients may have the following: Adrenal insufficiency (Addison disease), Diabetes mellitus type 2, Hepatomegaly, Hyperparathyroidism, Hypogonadism, Lymphadenopathy, Monoclonal gammopathy, Osteoclastic bone lesions, Polyneuropathy (peripheral motor and sensory manifestations), Primary hypothyroidism, Skin abnormalities (eg, hyperpigmentation, dermal thickening, hirsutism, angiomas, hypertrichosis). […] Other symptoms and signs of POEMS syndrome may include edema, ascites, pleural effusion, papilledema, and fever. Approximately 15% of patients with POEMS syndrome have associated Castleman disease (a lymphoproliferative disorder, some forms of which are associated with infection by HIV or human herpesvirus 8).

• #31 POEMS syndrome – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/poems-syndrome/symptoms-causes/syc-20352678

  POEMS syndrome is a rare blood disorder that damages nerves and affects other parts of the body. Symptoms may include weak legs, trouble breathing, a larger spleen or liver, and skin changes. […] Without treatment, POEMS syndrome gets worse quickly. It can become life-threatening. So early diagnosis is vital. […] The symptoms of POEMS syndrome make up its name. Symptoms most often appear over weeks to months. They include the following: Polyneuropathy. This may include numbness, tingling and weakness in the legs. In time, it might affect the hands. It also involves trouble breathing. Polyneuropathy must be present for a POEMS diagnosis. Organomegaly. This is an enlarged spleen, liver or lymph nodes. Endocrinopathy and edema. Atypical hormone levels can cause an underactive thyroid, also called hypothyroidism. The hormone levels also can cause diabetes, sexual issues, tiredness, and swelling in arms and legs. Monoclonal protein. This is a protein that atypical plasma cells in bone marrow make. The monoclonal protein goes into the bloodstream. This must be present for the diagnosis of POEMS syndrome. Monoclonal protein often is linked with bones getting harder or thicker. Skin changes. This may involve having more color than usual on the skin. There may be red spots, which may be harder to see on Black or brown skin. The skin might be thicker. There might be more hair on the face or the legs.

• #32 POEMS Syndrome – Endocrine and Metabolic Disorders – Merck Manual Professional Edition

  https://www.merckmanuals.com/professional/endocrine-and-metabolic-disorders/polyglandular-deficiency-syndromes/poems-syndrome

  POEMS syndrome is a rare paraneoplastic disorder and is probably caused by circulating immunoglobulins caused by a plasma cell disorder. […] Patients may have the following: Adrenal insufficiency (Addison disease), Diabetes mellitus type 2, Hepatomegaly, Hyperparathyroidism, Hypogonadism, Lymphadenopathy, Monoclonal gammopathy, Osteoclastic bone lesions, Polyneuropathy (peripheral motor and sensory manifestations), Primary hypothyroidism, Skin abnormalities (eg, hyperpigmentation, dermal thickening, hirsutism, angiomas, hypertrichosis). […] Other symptoms and signs of POEMS syndrome may include edema, ascites, pleural effusion, papilledema, and fever. Approximately 15% of patients with POEMS syndrome have associated Castleman disease (a lymphoproliferative disorder, some forms of which are associated with infection by HIV or human herpesvirus 8).

• #33 Endocrine Manifestations in POEMS Syndrome: a case report and literature review | BMC Endocrine Disorders | Full Text

  https://bmcendocrdisord.biomedcentral.com/articles/10.1186/s12902-019-0355-6

  The second most common abnormality was adrenal insufficiency, followed by hypothyroidism and subclinical hypothyroidism, then diabetes or glucose intolerance. Additionally, 27-45% of patients suffered from hypocalcemia. More than half had multiple axes involvement, including gonadal, thyroid, pancreas, and adrenal disorders. […] POEMS syndrome is a multisystem disease with a wide spectrum of clinical manifestations and progressive features, which makes its diagnosis a challenge and easily leads to misdiagnosis.

• #34 POEMS Syndrome: Background, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/1097031-overview

  POEMS syndrome is a rare multisystemic disease that occurs in the setting of a plasma cell dyscrasia. […] The polyneuropathy associated with POEMS syndrome is bilateral and symmetric. It involves both motor and sensory nerves, begins distally, and has a progressive proximal spread. Associated cranial or autonomic nerves are not involved. Both demyelination and axonal degeneration are noted. […] Multiple endocrinopathies have been associated with POEMS syndrome, and most patients have more than one endocrine abnormality. Many of the abnormalities noted can be explained by elevations in estrogen levels. Impotence and gynecomastia are common among men. Amenorrhea is common among women. Diabetes mellitus and glucose intolerance are also noted in many patients. Other associated endocrinopathies include hypothyroidism, hyperprolactinemia, and hypoparathyroidism.

• #35 POEMS Syndrome – Treatment abroad

  https://www.gsdinternational.com/conditions/poems-syndrome

  The onset symptoms are mainly associated with bilateral polyneuropathy, which affects both motor and sensory fibers of the lower extremities and manifests itself as muscle weakness in the arms and legs, tingling, numbness, burning pain and a lack of sensitivity. […] Organomegaly, that is, an increase in the size of some internal organs, most often lymph nodes, liver, spleen and, less often, heart, can be found in about half of patients. Among the endocrinological changes, the most common are hypothyroidism and adrenal insufficiency; Also, sexual function is often involved with an increase in the level of estrogen in the blood, which determines impotence and gynecomastia in men, that is, excessive development of the mammary glands, and in women – the absence of menstruation and hypertrichosis. Also common: diabetes / glucose intolerance.

• #36 POEMS syndrome – Wikipedia

  https://en.wikipedia.org/wiki/POEMS_syndrome

  Papilledema, polyneuropathy, edema, hyperpigmentation, hypogonadism, visceromegaly, amenorrhea, gynecomastia, erectile dysfunction, and testicular atrophy. […] The signs and symptoms of POEMS syndrome are highly variable. This often leads to long delays (e.g. 13-18 months) between the onset of initial symptoms and diagnosis. […] POEMS syndrome typically begins in middle age the average age at onset is 50 and affects up to twice as many men as women. […] Subacute, distal, symmetrical sensorimotor neuropathy with allodynia and hyperpathia is the most frequent presentation of POEMS syndrome. Neuropathy is often the first trait, and it may be the only initial symptom. […] Approximately 84% of POEMS syndrome patients have characteristics of several endocrinopathies. Hypogonadism is the most prevalent endocrine disorder, followed by thyroid anomalies, glucose metabolism defects, and adrenal insufficiency.

• #37 POEMS syndrome | University of Iowa Health Care

  https://uihc.org/health-topics/poems-syndrome

  POEMS syndrome is an extremely rare blood disorder that affects several body systems. […] POEMS syndrome patients experience numbness, tingling, and weakness in the toes, feet, and later the hands. […] POEMS syndrome patients may have an underactive thyroid, metabolism issues, diabetes, or improper functioning of the ovaries or testes. […] POEMS syndrome patients may experience tumors known as plasmacytomas that appear on x-rays as thickening of the bone. […] Skin abnormalities may include darkening of the skin, hardening or thickening of the skin, increased facial or leg hair, and excessive sweating. […] As the condition progresses it can lead to problems in the lungs, kidneys, and heart. […] POEMS syndrome patients can be effectively treated and return to an overall normal quality of life.

• #38 POEMS syndrome – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/poems-syndrome/symptoms-causes/syc-20352678

  POEMS syndrome is a rare blood disorder that damages nerves and affects other parts of the body. Symptoms may include weak legs, trouble breathing, a larger spleen or liver, and skin changes. […] Without treatment, POEMS syndrome gets worse quickly. It can become life-threatening. So early diagnosis is vital. […] The symptoms of POEMS syndrome make up its name. Symptoms most often appear over weeks to months. They include the following: Polyneuropathy. This may include numbness, tingling and weakness in the legs. In time, it might affect the hands. It also involves trouble breathing. Polyneuropathy must be present for a POEMS diagnosis. Organomegaly. This is an enlarged spleen, liver or lymph nodes. Endocrinopathy and edema. Atypical hormone levels can cause an underactive thyroid, also called hypothyroidism. The hormone levels also can cause diabetes, sexual issues, tiredness, and swelling in arms and legs. Monoclonal protein. This is a protein that atypical plasma cells in bone marrow make. The monoclonal protein goes into the bloodstream. This must be present for the diagnosis of POEMS syndrome. Monoclonal protein often is linked with bones getting harder or thicker. Skin changes. This may involve having more color than usual on the skin. There may be red spots, which may be harder to see on Black or brown skin. The skin might be thicker. There might be more hair on the face or the legs.

• #39 POEMS Syndrome: 2019 Update on diagnosis, risk-stratification, and management – PubMed

  https://pubmed.ncbi.nlm.nih.gov/31012139/

  Polyneuropathy, organomegaly, endocrinopathy, M-protein, skin changes (POEMS) syndrome is a paraneoplastic syndrome due to an underlying plasma cell neoplasm. The major criteria for the syndrome are polyradiculoneuropathy, clonal plasma cell disorder (PCD), sclerotic bone lesions, elevated vascular endothelial growth factor, and the presence of Castleman disease. Minor features include organomegaly, endocrinopathy, characteristic skin changes, papilledema, extravascular volume overload, and thrombocytosis. […] The diagnosis of POEMS syndrome is made with three of the major criteria, two of which must include polyradiculoneuropathy and clonal PCD, and at least one of the minor criteria. […] Risk factors include low serum albumin, age, pleural effusion, pulmonary hypertension, and reduced eGFR.

• #40 POEMS syndrome – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/poems-syndrome/symptoms-causes/syc-20352678

  POEMS syndrome is a rare blood disorder that damages nerves and affects other parts of the body. Symptoms may include weak legs, trouble breathing, a larger spleen or liver, and skin changes. […] Without treatment, POEMS syndrome gets worse quickly. It can become life-threatening. So early diagnosis is vital. […] The symptoms of POEMS syndrome make up its name. Symptoms most often appear over weeks to months. They include the following: Polyneuropathy. This may include numbness, tingling and weakness in the legs. In time, it might affect the hands. It also involves trouble breathing. Polyneuropathy must be present for a POEMS diagnosis. Organomegaly. This is an enlarged spleen, liver or lymph nodes. Endocrinopathy and edema. Atypical hormone levels can cause an underactive thyroid, also called hypothyroidism. The hormone levels also can cause diabetes, sexual issues, tiredness, and swelling in arms and legs. Monoclonal protein. This is a protein that atypical plasma cells in bone marrow make. The monoclonal protein goes into the bloodstream. This must be present for the diagnosis of POEMS syndrome. Monoclonal protein often is linked with bones getting harder or thicker. Skin changes. This may involve having more color than usual on the skin. There may be red spots, which may be harder to see on Black or brown skin. The skin might be thicker. There might be more hair on the face or the legs.

• #41 Comprehensive Diagnosis and Management of POEMS Syndrome

  https://pmc.ncbi.nlm.nih.gov/articles/PMC9624442/

  These clinical features alongside a monoclonal plasma cell disorderalmost always lambda light-chain restrictedor unexplained polycythemia or thrombocytosis should prompt investigation for other POEMS features. Peripheral edema is found in most and may present as ascites (7%54%), pleural (3%43%), and pericardial effusions (1%64%). This is often poorly responsive to diuretics as the excess fluid is not within the vascular compartment. […] Skin lesions are common (70%). Glomerular hemangiomata are a pathognomonic finding, but sclerodermoid changes of the digits (5%43%), temperature-sensitive blue/purple discoloration of the feet (acrocyanosis 19%), leukonychia, clubbing (5%49%), hyperpigmentation of the skin (46%93%), and new growth of excess bodily hair (26%74%) are frequent and readily visible.

• #42

  https://journals.lww.com/hemasphere/fulltext/2022/11000/comprehensive_diagnosis_and_management_of_poems.12.aspx

  These clinical features alongside a monoclonal plasma cell disorderalmost always lambda light-chain restrictedor unexplained polycythemia or thrombocytosis should prompt investigation for other POEMS features. Peripheral edema is found in most and may present as ascites (7%54%), pleural (3%43%), and pericardial effusions (1%64%). This is often poorly responsive to diuretics as the excess fluid is not within the vascular compartment. Papilledema, sometimes with characteristic subretinal hemorrhages, and without raised intracranial pressure is present in 30%40% of patients. Stroke is a recognized feature, and noninfiltrative, noninfective pachymeningitis caused by meningeal fluid leak has been recently reported in 70% of cases. […] Skin lesions are common (70%). Glomerular hemangiomata are a pathognomonic finding, but sclerodermoid changes of the digits (5%43%), temperature-sensitive blue/purple discoloration of the feet (acrocyanosis 19%), leukonychia, clubbing (5%49%), hyperpigmentation of the skin (46%93%), and new growth of excess bodily hair (26%74%) are frequent and readily visible. Other features suggestive of POEMS include fatigue, unexplained weight loss, enlarged lymph nodes or organomegaly, unprovoked venous and/or arterial thrombosis and features of gonadal failure (erectile dysfunction, early menopausal symptoms, infertility).

• #43 POEMS syndrome – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/poems-syndrome/symptoms-causes/syc-20352678

  POEMS syndrome is a rare blood disorder that damages nerves and affects other parts of the body. Symptoms may include weak legs, trouble breathing, a larger spleen or liver, and skin changes. […] Without treatment, POEMS syndrome gets worse quickly. It can become life-threatening. So early diagnosis is vital. […] The symptoms of POEMS syndrome make up its name. Symptoms most often appear over weeks to months. They include the following: Polyneuropathy. This may include numbness, tingling and weakness in the legs. In time, it might affect the hands. It also involves trouble breathing. Polyneuropathy must be present for a POEMS diagnosis. Organomegaly. This is an enlarged spleen, liver or lymph nodes. Endocrinopathy and edema. Atypical hormone levels can cause an underactive thyroid, also called hypothyroidism. The hormone levels also can cause diabetes, sexual issues, tiredness, and swelling in arms and legs. Monoclonal protein. This is a protein that atypical plasma cells in bone marrow make. The monoclonal protein goes into the bloodstream. This must be present for the diagnosis of POEMS syndrome. Monoclonal protein often is linked with bones getting harder or thicker. Skin changes. This may involve having more color than usual on the skin. There may be red spots, which may be harder to see on Black or brown skin. The skin might be thicker. There might be more hair on the face or the legs.

• #44 POEMS Syndrome: What It Is, Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/17985-poems-syndrome

  Imaging procedures may show that you have an enlarged: Liver (hepatomegaly). Spleen (splenomegaly). Lymph nodes (lymphadenopathy). […] Low levels of sex hormones are the most common sign of endocrinopathy with POEMS. […] High levels of M-protein are a sign of POEMS. Too much M-protein can cause tumors to form in your blood. Excessive M-protein can cause your bones to thicken or harden (osteosclerosis). […] A variety of skin changes may take place, including: Darker skin. Thicker skin. White fingernails. Increased hair growth on your face and legs. Growth of blood vessels (particularly on your chest) that look like small cherries. […] Other symptoms may include: Swelling in your arms and legs. Unexplained weight loss. Bone and joint pain. Vision problems. Trouble breathing. Fatigue. Fever.

• #45

  https://link.springer.com/article/10.1007/s00415-018-9110-6

  Many patients quickly become wheelchair- or bed-bound due to weakness or pain. […] Electrodiagnostic studies demonstrate a length-dependent sensorimotor neuropathy, typically demyelinating, but with axonal degeneration. […] In a study of 51 patients with POEMS syndrome, 70% met the European Federation of Neurological Societies and Peripheral Nerve Society criteria for definite chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). […] Clinical examination may reveal distal wasting, weakness and sensory loss affecting both large and small fibre sensory modalities. […] Patients with POEMS syndrome typically have few monoclonal plasma cells on iliac crest biopsies. […] In a study of 87 patients at the Mayo clinic, the median percentage of plasma cells in 67 pre-treatment bone marrow specimens was 5%, with monoclonal plasma cells detected in two-thirds. […] Overall survival is dependent on the presence or absence of CD, osteosclerotic lesions, and POEMS syndrome. […] With the right treatment, however, prognosis in many patients can be very good.

• #46

  https://journals.lww.com/hemasphere/fulltext/2022/11000/comprehensive_diagnosis_and_management_of_poems.12.aspx

  These clinical features alongside a monoclonal plasma cell disorderalmost always lambda light-chain restrictedor unexplained polycythemia or thrombocytosis should prompt investigation for other POEMS features. Peripheral edema is found in most and may present as ascites (7%54%), pleural (3%43%), and pericardial effusions (1%64%). This is often poorly responsive to diuretics as the excess fluid is not within the vascular compartment. Papilledema, sometimes with characteristic subretinal hemorrhages, and without raised intracranial pressure is present in 30%40% of patients. Stroke is a recognized feature, and noninfiltrative, noninfective pachymeningitis caused by meningeal fluid leak has been recently reported in 70% of cases. […] Skin lesions are common (70%). Glomerular hemangiomata are a pathognomonic finding, but sclerodermoid changes of the digits (5%43%), temperature-sensitive blue/purple discoloration of the feet (acrocyanosis 19%), leukonychia, clubbing (5%49%), hyperpigmentation of the skin (46%93%), and new growth of excess bodily hair (26%74%) are frequent and readily visible. Other features suggestive of POEMS include fatigue, unexplained weight loss, enlarged lymph nodes or organomegaly, unprovoked venous and/or arterial thrombosis and features of gonadal failure (erectile dysfunction, early menopausal symptoms, infertility).

• #47

  https://journals.lww.com/hemasphere/fulltext/2022/11000/comprehensive_diagnosis_and_management_of_poems.12.aspx

  These clinical features alongside a monoclonal plasma cell disorderalmost always lambda light-chain restrictedor unexplained polycythemia or thrombocytosis should prompt investigation for other POEMS features. Peripheral edema is found in most and may present as ascites (7%54%), pleural (3%43%), and pericardial effusions (1%64%). This is often poorly responsive to diuretics as the excess fluid is not within the vascular compartment. Papilledema, sometimes with characteristic subretinal hemorrhages, and without raised intracranial pressure is present in 30%40% of patients. Stroke is a recognized feature, and noninfiltrative, noninfective pachymeningitis caused by meningeal fluid leak has been recently reported in 70% of cases. […] Skin lesions are common (70%). Glomerular hemangiomata are a pathognomonic finding, but sclerodermoid changes of the digits (5%43%), temperature-sensitive blue/purple discoloration of the feet (acrocyanosis 19%), leukonychia, clubbing (5%49%), hyperpigmentation of the skin (46%93%), and new growth of excess bodily hair (26%74%) are frequent and readily visible. Other features suggestive of POEMS include fatigue, unexplained weight loss, enlarged lymph nodes or organomegaly, unprovoked venous and/or arterial thrombosis and features of gonadal failure (erectile dysfunction, early menopausal symptoms, infertility).

• #48 POEMS syndrome – Wikipedia

  https://en.wikipedia.org/wiki/POEMS_syndrome

  About 90-100% of those with POEMS syndrome will experience skin changes. The most common manifestations are hyperpigmentation and haemangiomas. […] Papilledema is often one of the earliest signs of POEMS disease and is usually bilateral. […] The diagnosis of POEMS syndrome is based on meeting its two mandatory criteria, meeting at least one of its 3 other major criteria, and meeting at least one of its 6 minor criteria. […] As reported by Dispenzieri et al. Mayo Clinic treatment regimens are tailored to treat the clinical manifestations and prognosis for the rate of progression of the POEMS syndrome in each patient. […] In 59 patients treated with the chemotherapy/transplantation regimen, the Mayo Clinic reported progression-free survival rates of 98%, 94%, and 75% at 1, 2, and 5 years, respectively.

• #49

  https://journals.lww.com/hemasphere/fulltext/2022/11000/comprehensive_diagnosis_and_management_of_poems.12.aspx

  These clinical features alongside a monoclonal plasma cell disorderalmost always lambda light-chain restrictedor unexplained polycythemia or thrombocytosis should prompt investigation for other POEMS features. Peripheral edema is found in most and may present as ascites (7%54%), pleural (3%43%), and pericardial effusions (1%64%). This is often poorly responsive to diuretics as the excess fluid is not within the vascular compartment. Papilledema, sometimes with characteristic subretinal hemorrhages, and without raised intracranial pressure is present in 30%40% of patients. Stroke is a recognized feature, and noninfiltrative, noninfective pachymeningitis caused by meningeal fluid leak has been recently reported in 70% of cases. […] Skin lesions are common (70%). Glomerular hemangiomata are a pathognomonic finding, but sclerodermoid changes of the digits (5%43%), temperature-sensitive blue/purple discoloration of the feet (acrocyanosis 19%), leukonychia, clubbing (5%49%), hyperpigmentation of the skin (46%93%), and new growth of excess bodily hair (26%74%) are frequent and readily visible. Other features suggestive of POEMS include fatigue, unexplained weight loss, enlarged lymph nodes or organomegaly, unprovoked venous and/or arterial thrombosis and features of gonadal failure (erectile dysfunction, early menopausal symptoms, infertility).

• #50

  https://journals.lww.com/hemasphere/fulltext/2022/11000/comprehensive_diagnosis_and_management_of_poems.12.aspx

  These clinical features alongside a monoclonal plasma cell disorderalmost always lambda light-chain restrictedor unexplained polycythemia or thrombocytosis should prompt investigation for other POEMS features. Peripheral edema is found in most and may present as ascites (7%54%), pleural (3%43%), and pericardial effusions (1%64%). This is often poorly responsive to diuretics as the excess fluid is not within the vascular compartment. Papilledema, sometimes with characteristic subretinal hemorrhages, and without raised intracranial pressure is present in 30%40% of patients. Stroke is a recognized feature, and noninfiltrative, noninfective pachymeningitis caused by meningeal fluid leak has been recently reported in 70% of cases. […] Skin lesions are common (70%). Glomerular hemangiomata are a pathognomonic finding, but sclerodermoid changes of the digits (5%43%), temperature-sensitive blue/purple discoloration of the feet (acrocyanosis 19%), leukonychia, clubbing (5%49%), hyperpigmentation of the skin (46%93%), and new growth of excess bodily hair (26%74%) are frequent and readily visible. Other features suggestive of POEMS include fatigue, unexplained weight loss, enlarged lymph nodes or organomegaly, unprovoked venous and/or arterial thrombosis and features of gonadal failure (erectile dysfunction, early menopausal symptoms, infertility).

• #51

  https://journals.lww.com/hemasphere/fulltext/2022/11000/comprehensive_diagnosis_and_management_of_poems.12.aspx

  These clinical features alongside a monoclonal plasma cell disorderalmost always lambda light-chain restrictedor unexplained polycythemia or thrombocytosis should prompt investigation for other POEMS features. Peripheral edema is found in most and may present as ascites (7%54%), pleural (3%43%), and pericardial effusions (1%64%). This is often poorly responsive to diuretics as the excess fluid is not within the vascular compartment. Papilledema, sometimes with characteristic subretinal hemorrhages, and without raised intracranial pressure is present in 30%40% of patients. Stroke is a recognized feature, and noninfiltrative, noninfective pachymeningitis caused by meningeal fluid leak has been recently reported in 70% of cases. […] Skin lesions are common (70%). Glomerular hemangiomata are a pathognomonic finding, but sclerodermoid changes of the digits (5%43%), temperature-sensitive blue/purple discoloration of the feet (acrocyanosis 19%), leukonychia, clubbing (5%49%), hyperpigmentation of the skin (46%93%), and new growth of excess bodily hair (26%74%) are frequent and readily visible. Other features suggestive of POEMS include fatigue, unexplained weight loss, enlarged lymph nodes or organomegaly, unprovoked venous and/or arterial thrombosis and features of gonadal failure (erectile dysfunction, early menopausal symptoms, infertility).

• #52

  https://journals.lww.com/hemasphere/fulltext/2022/11000/comprehensive_diagnosis_and_management_of_poems.12.aspx

  These clinical features alongside a monoclonal plasma cell disorderalmost always lambda light-chain restrictedor unexplained polycythemia or thrombocytosis should prompt investigation for other POEMS features. Peripheral edema is found in most and may present as ascites (7%54%), pleural (3%43%), and pericardial effusions (1%64%). This is often poorly responsive to diuretics as the excess fluid is not within the vascular compartment. Papilledema, sometimes with characteristic subretinal hemorrhages, and without raised intracranial pressure is present in 30%40% of patients. Stroke is a recognized feature, and noninfiltrative, noninfective pachymeningitis caused by meningeal fluid leak has been recently reported in 70% of cases. […] Skin lesions are common (70%). Glomerular hemangiomata are a pathognomonic finding, but sclerodermoid changes of the digits (5%43%), temperature-sensitive blue/purple discoloration of the feet (acrocyanosis 19%), leukonychia, clubbing (5%49%), hyperpigmentation of the skin (46%93%), and new growth of excess bodily hair (26%74%) are frequent and readily visible. Other features suggestive of POEMS include fatigue, unexplained weight loss, enlarged lymph nodes or organomegaly, unprovoked venous and/or arterial thrombosis and features of gonadal failure (erectile dysfunction, early menopausal symptoms, infertility).

• #53

  https://journals.lww.com/hemasphere/fulltext/2022/11000/comprehensive_diagnosis_and_management_of_poems.12.aspx

  These clinical features alongside a monoclonal plasma cell disorderalmost always lambda light-chain restrictedor unexplained polycythemia or thrombocytosis should prompt investigation for other POEMS features. Peripheral edema is found in most and may present as ascites (7%54%), pleural (3%43%), and pericardial effusions (1%64%). This is often poorly responsive to diuretics as the excess fluid is not within the vascular compartment. Papilledema, sometimes with characteristic subretinal hemorrhages, and without raised intracranial pressure is present in 30%40% of patients. Stroke is a recognized feature, and noninfiltrative, noninfective pachymeningitis caused by meningeal fluid leak has been recently reported in 70% of cases. […] Skin lesions are common (70%). Glomerular hemangiomata are a pathognomonic finding, but sclerodermoid changes of the digits (5%43%), temperature-sensitive blue/purple discoloration of the feet (acrocyanosis 19%), leukonychia, clubbing (5%49%), hyperpigmentation of the skin (46%93%), and new growth of excess bodily hair (26%74%) are frequent and readily visible. Other features suggestive of POEMS include fatigue, unexplained weight loss, enlarged lymph nodes or organomegaly, unprovoked venous and/or arterial thrombosis and features of gonadal failure (erectile dysfunction, early menopausal symptoms, infertility).

• #54

  https://journals.lww.com/hemasphere/fulltext/2022/11000/comprehensive_diagnosis_and_management_of_poems.12.aspx

  These clinical features alongside a monoclonal plasma cell disorderalmost always lambda light-chain restrictedor unexplained polycythemia or thrombocytosis should prompt investigation for other POEMS features. Peripheral edema is found in most and may present as ascites (7%54%), pleural (3%43%), and pericardial effusions (1%64%). This is often poorly responsive to diuretics as the excess fluid is not within the vascular compartment. Papilledema, sometimes with characteristic subretinal hemorrhages, and without raised intracranial pressure is present in 30%40% of patients. Stroke is a recognized feature, and noninfiltrative, noninfective pachymeningitis caused by meningeal fluid leak has been recently reported in 70% of cases. […] Skin lesions are common (70%). Glomerular hemangiomata are a pathognomonic finding, but sclerodermoid changes of the digits (5%43%), temperature-sensitive blue/purple discoloration of the feet (acrocyanosis 19%), leukonychia, clubbing (5%49%), hyperpigmentation of the skin (46%93%), and new growth of excess bodily hair (26%74%) are frequent and readily visible. Other features suggestive of POEMS include fatigue, unexplained weight loss, enlarged lymph nodes or organomegaly, unprovoked venous and/or arterial thrombosis and features of gonadal failure (erectile dysfunction, early menopausal symptoms, infertility).

• #55

  https://journals.lww.com/hemasphere/fulltext/2022/11000/comprehensive_diagnosis_and_management_of_poems.12.aspx

  These clinical features alongside a monoclonal plasma cell disorderalmost always lambda light-chain restrictedor unexplained polycythemia or thrombocytosis should prompt investigation for other POEMS features. Peripheral edema is found in most and may present as ascites (7%54%), pleural (3%43%), and pericardial effusions (1%64%). This is often poorly responsive to diuretics as the excess fluid is not within the vascular compartment. Papilledema, sometimes with characteristic subretinal hemorrhages, and without raised intracranial pressure is present in 30%40% of patients. Stroke is a recognized feature, and noninfiltrative, noninfective pachymeningitis caused by meningeal fluid leak has been recently reported in 70% of cases. […] Skin lesions are common (70%). Glomerular hemangiomata are a pathognomonic finding, but sclerodermoid changes of the digits (5%43%), temperature-sensitive blue/purple discoloration of the feet (acrocyanosis 19%), leukonychia, clubbing (5%49%), hyperpigmentation of the skin (46%93%), and new growth of excess bodily hair (26%74%) are frequent and readily visible. Other features suggestive of POEMS include fatigue, unexplained weight loss, enlarged lymph nodes or organomegaly, unprovoked venous and/or arterial thrombosis and features of gonadal failure (erectile dysfunction, early menopausal symptoms, infertility).

• #56 POEMS Syndrome | Condition | UAMS Health

  https://uamshealth.com/condition/poems-syndrome/

  Polyneuropathy is characterized by chronic, progressive disease affecting the peripheral nervous system. Individuals with POEMS syndrome experience numbness, tingling, feelings of coldness, and weakness, starting in the toes and feet and progressively working its way upwards. Over time, the hands can be affected. […] Organomegaly refers to abnormal enlargement of the spleen, liver and lymph nodes. Swelling, especially of the lymph nodes, may be present. […] Endocrinopathy refers to abnormalities in the endocrine system (the network of glands that secrete hormones into the circulatory system). Abnormal hormone levels can lead to hypothyroidism (underactive thyroid), metabolism problems, improper functioning of the ovaries in females or testes in males, and diabetes. […] Skin abnormalities in POEMS syndrome can include abnormal darkening of the skin, hardening and thickening of the skin (sclerosis), increased facial or leg hair, and excessive sweating.

• #57 POEMS Syndrome: Symptoms, Causes, & Treatments

  https://www.medicoverhospitals.in/diseases/poems-syndrome/

  Peripheral neuropathy is a hallmark feature and often the earliest symptom of POEMS syndrome. Patients typically experience sensory and motor deficits, presenting as numbness, tingling, and weakness in the extremities. This progressive neuropathy can significantly impact the quality of life and daily functioning. […] Enlargement of organs, particularly the liver, spleen, and lymph nodes, is common in POEMS syndrome. This organomegaly can lead to abdominal discomfort and other systemic complications. […] Endocrine abnormalities are frequent, with patients often exhibiting symptoms of hypothyroidism, diabetes mellitus, adrenal insufficiency, or gonadal dysfunction. These hormonal imbalances contribute to the complexity of managing the syndrome. […] Dermatological manifestations, such as hyperpigmentation, skin thickening, and hemangiomas, are often observed. These changes can serve as valuable clinical clues in the diagnostic process.

• #58 POEMS syndrome – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/poems-syndrome/symptoms-causes/syc-20352678

  Not all the symptoms of POEMS syndrome are in the name. And not everyone with POEMS syndrome has all the symptoms in the name. But everyone with POEMS syndrome has polyneuropathy and monoclonal protein. […] Other symptoms some people with POEMS syndrome have spell out PEST. PEST stands for: Papilledema. This is swelling in the back of the eyes. Extravascular volume overload. This might include ankle swelling, fluid between the lung and the ribs, or fluid in the belly. Sclerotic bone lesions. X-rays can show these bone spots. They’re most often not painful. Thrombocytosis and erythrocytosis. These are higher levels of red cells and platelets in the bloodstream. […] Make an appointment with your healthcare professional if you have any of the symptoms of POEMS syndrome. […] The outlook for people with POEMS syndrome has improved greatly over the years.

• #59 A Review of POEMS Syndrome

  https://www.cancernetwork.com/view/review-poems-syndrome

  The presence of a monoclonal plasma cell disorder is required to make the diagnosis. […] Organomegaly manifests commonly as hepatomegaly, splenomegaly, and/or lymphadenopathy. […] The endocrine features are diverse and may involve any endocrine gland function, hypothalamic function, or pituitary function. Erectile dysfunction is a common first or second symptom in male patients with POEMS. […] Skin manifestations include hyperpigmentation, a recent outcropping of hemangiomas, hypertrichosis, dependent rubor and acrocyanosis, white nails, sclerodermoid changes, flushing, or nail clubbing. […] Papilledema is present in at least one-third of patients. […] Extravascular overload most commonly manifests as peripheral edema, but pleural effusion, ascites, and pericardial effusions are also common.

• #60 A Review of POEMS Syndrome

  https://www.cancernetwork.com/view/review-poems-syndrome

  The presence of a monoclonal plasma cell disorder is required to make the diagnosis. […] Organomegaly manifests commonly as hepatomegaly, splenomegaly, and/or lymphadenopathy. […] The endocrine features are diverse and may involve any endocrine gland function, hypothalamic function, or pituitary function. Erectile dysfunction is a common first or second symptom in male patients with POEMS. […] Skin manifestations include hyperpigmentation, a recent outcropping of hemangiomas, hypertrichosis, dependent rubor and acrocyanosis, white nails, sclerodermoid changes, flushing, or nail clubbing. […] Papilledema is present in at least one-third of patients. […] Extravascular overload most commonly manifests as peripheral edema, but pleural effusion, ascites, and pericardial effusions are also common.

• #61 POEMS syndrome – Wikipedia

  https://en.wikipedia.org/wiki/POEMS_syndrome

  Papilledema, polyneuropathy, edema, hyperpigmentation, hypogonadism, visceromegaly, amenorrhea, gynecomastia, erectile dysfunction, and testicular atrophy. […] The signs and symptoms of POEMS syndrome are highly variable. This often leads to long delays (e.g. 13-18 months) between the onset of initial symptoms and diagnosis. […] POEMS syndrome typically begins in middle age the average age at onset is 50 and affects up to twice as many men as women. […] Subacute, distal, symmetrical sensorimotor neuropathy with allodynia and hyperpathia is the most frequent presentation of POEMS syndrome. Neuropathy is often the first trait, and it may be the only initial symptom. […] Approximately 84% of POEMS syndrome patients have characteristics of several endocrinopathies. Hypogonadism is the most prevalent endocrine disorder, followed by thyroid anomalies, glucose metabolism defects, and adrenal insufficiency.

• #62 A Review of POEMS Syndrome

  https://www.cancernetwork.com/view/review-poems-syndrome

  The presence of a monoclonal plasma cell disorder is required to make the diagnosis. […] Organomegaly manifests commonly as hepatomegaly, splenomegaly, and/or lymphadenopathy. […] The endocrine features are diverse and may involve any endocrine gland function, hypothalamic function, or pituitary function. Erectile dysfunction is a common first or second symptom in male patients with POEMS. […] Skin manifestations include hyperpigmentation, a recent outcropping of hemangiomas, hypertrichosis, dependent rubor and acrocyanosis, white nails, sclerodermoid changes, flushing, or nail clubbing. […] Papilledema is present in at least one-third of patients. […] Extravascular overload most commonly manifests as peripheral edema, but pleural effusion, ascites, and pericardial effusions are also common.

• #63 Comprehensive Diagnosis and Management of POEMS Syndrome

  https://pmc.ncbi.nlm.nih.gov/articles/PMC9624442/

  These clinical features alongside a monoclonal plasma cell disorderalmost always lambda light-chain restrictedor unexplained polycythemia or thrombocytosis should prompt investigation for other POEMS features. Peripheral edema is found in most and may present as ascites (7%54%), pleural (3%43%), and pericardial effusions (1%64%). This is often poorly responsive to diuretics as the excess fluid is not within the vascular compartment. […] Skin lesions are common (70%). Glomerular hemangiomata are a pathognomonic finding, but sclerodermoid changes of the digits (5%43%), temperature-sensitive blue/purple discoloration of the feet (acrocyanosis 19%), leukonychia, clubbing (5%49%), hyperpigmentation of the skin (46%93%), and new growth of excess bodily hair (26%74%) are frequent and readily visible.

• #64

  https://journals.lww.com/hemasphere/fulltext/2022/11000/comprehensive_diagnosis_and_management_of_poems.12.aspx

  These clinical features alongside a monoclonal plasma cell disorderalmost always lambda light-chain restrictedor unexplained polycythemia or thrombocytosis should prompt investigation for other POEMS features. Peripheral edema is found in most and may present as ascites (7%54%), pleural (3%43%), and pericardial effusions (1%64%). This is often poorly responsive to diuretics as the excess fluid is not within the vascular compartment. Papilledema, sometimes with characteristic subretinal hemorrhages, and without raised intracranial pressure is present in 30%40% of patients. Stroke is a recognized feature, and noninfiltrative, noninfective pachymeningitis caused by meningeal fluid leak has been recently reported in 70% of cases. […] Skin lesions are common (70%). Glomerular hemangiomata are a pathognomonic finding, but sclerodermoid changes of the digits (5%43%), temperature-sensitive blue/purple discoloration of the feet (acrocyanosis 19%), leukonychia, clubbing (5%49%), hyperpigmentation of the skin (46%93%), and new growth of excess bodily hair (26%74%) are frequent and readily visible. Other features suggestive of POEMS include fatigue, unexplained weight loss, enlarged lymph nodes or organomegaly, unprovoked venous and/or arterial thrombosis and features of gonadal failure (erectile dysfunction, early menopausal symptoms, infertility).

• #65

  https://journals.lww.com/hemasphere/fulltext/2022/11000/comprehensive_diagnosis_and_management_of_poems.12.aspx

  These clinical features alongside a monoclonal plasma cell disorderalmost always lambda light-chain restrictedor unexplained polycythemia or thrombocytosis should prompt investigation for other POEMS features. Peripheral edema is found in most and may present as ascites (7%54%), pleural (3%43%), and pericardial effusions (1%64%). This is often poorly responsive to diuretics as the excess fluid is not within the vascular compartment. Papilledema, sometimes with characteristic subretinal hemorrhages, and without raised intracranial pressure is present in 30%40% of patients. Stroke is a recognized feature, and noninfiltrative, noninfective pachymeningitis caused by meningeal fluid leak has been recently reported in 70% of cases. […] Skin lesions are common (70%). Glomerular hemangiomata are a pathognomonic finding, but sclerodermoid changes of the digits (5%43%), temperature-sensitive blue/purple discoloration of the feet (acrocyanosis 19%), leukonychia, clubbing (5%49%), hyperpigmentation of the skin (46%93%), and new growth of excess bodily hair (26%74%) are frequent and readily visible. Other features suggestive of POEMS include fatigue, unexplained weight loss, enlarged lymph nodes or organomegaly, unprovoked venous and/or arterial thrombosis and features of gonadal failure (erectile dysfunction, early menopausal symptoms, infertility).

• #66

  https://journals.lww.com/hemasphere/fulltext/2022/11000/comprehensive_diagnosis_and_management_of_poems.12.aspx

  These clinical features alongside a monoclonal plasma cell disorderalmost always lambda light-chain restrictedor unexplained polycythemia or thrombocytosis should prompt investigation for other POEMS features. Peripheral edema is found in most and may present as ascites (7%54%), pleural (3%43%), and pericardial effusions (1%64%). This is often poorly responsive to diuretics as the excess fluid is not within the vascular compartment. Papilledema, sometimes with characteristic subretinal hemorrhages, and without raised intracranial pressure is present in 30%40% of patients. Stroke is a recognized feature, and noninfiltrative, noninfective pachymeningitis caused by meningeal fluid leak has been recently reported in 70% of cases. […] Skin lesions are common (70%). Glomerular hemangiomata are a pathognomonic finding, but sclerodermoid changes of the digits (5%43%), temperature-sensitive blue/purple discoloration of the feet (acrocyanosis 19%), leukonychia, clubbing (5%49%), hyperpigmentation of the skin (46%93%), and new growth of excess bodily hair (26%74%) are frequent and readily visible. Other features suggestive of POEMS include fatigue, unexplained weight loss, enlarged lymph nodes or organomegaly, unprovoked venous and/or arterial thrombosis and features of gonadal failure (erectile dysfunction, early menopausal symptoms, infertility).

• #67

  https://journals.lww.com/hemasphere/fulltext/2022/11000/comprehensive_diagnosis_and_management_of_poems.12.aspx

  These clinical features alongside a monoclonal plasma cell disorderalmost always lambda light-chain restrictedor unexplained polycythemia or thrombocytosis should prompt investigation for other POEMS features. Peripheral edema is found in most and may present as ascites (7%54%), pleural (3%43%), and pericardial effusions (1%64%). This is often poorly responsive to diuretics as the excess fluid is not within the vascular compartment. Papilledema, sometimes with characteristic subretinal hemorrhages, and without raised intracranial pressure is present in 30%40% of patients. Stroke is a recognized feature, and noninfiltrative, noninfective pachymeningitis caused by meningeal fluid leak has been recently reported in 70% of cases. […] Skin lesions are common (70%). Glomerular hemangiomata are a pathognomonic finding, but sclerodermoid changes of the digits (5%43%), temperature-sensitive blue/purple discoloration of the feet (acrocyanosis 19%), leukonychia, clubbing (5%49%), hyperpigmentation of the skin (46%93%), and new growth of excess bodily hair (26%74%) are frequent and readily visible. Other features suggestive of POEMS include fatigue, unexplained weight loss, enlarged lymph nodes or organomegaly, unprovoked venous and/or arterial thrombosis and features of gonadal failure (erectile dysfunction, early menopausal symptoms, infertility).

• #68 A Review of POEMS Syndrome

  https://www.cancernetwork.com/view/review-poems-syndrome

  Patients with POEMS syndrome are at increased risk for arterial and/or venous thromboses during the course of their disease, with nearly 20% of patients experiencing one of these complications. […] The course of POEMS syndrome is usually chronic, with a median patient survival time of nearly 14 years. […] Only fingernail clubbing, extravascular volume overload (effusions, edema, and ascites), and respiratory symptoms have been associated with a significantly shorter overall survival time. […] The number of POEMS features does not affect survival. […] Once there is disseminated bone marrow involvement, albeit even in patients with a low plasma cell percentage, radiation is not expected to be curative. […] It is imperative that the treating physician (and patient) realize that there is a lag between completion of successful therapy and neurologic response, often with no discernible improvement until 3 to 6 months after completion of therapy. Maximal response is not seen until 2 to 3 years afterwards. Other features, such as anasarca, papilledema, and even skin changes, typically improve sooner.

• #69 POEMS syndrome – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/poems-syndrome/symptoms-causes/syc-20352678

  Not all the symptoms of POEMS syndrome are in the name. And not everyone with POEMS syndrome has all the symptoms in the name. But everyone with POEMS syndrome has polyneuropathy and monoclonal protein. […] Other symptoms some people with POEMS syndrome have spell out PEST. PEST stands for: Papilledema. This is swelling in the back of the eyes. Extravascular volume overload. This might include ankle swelling, fluid between the lung and the ribs, or fluid in the belly. Sclerotic bone lesions. X-rays can show these bone spots. They’re most often not painful. Thrombocytosis and erythrocytosis. These are higher levels of red cells and platelets in the bloodstream. […] Make an appointment with your healthcare professional if you have any of the symptoms of POEMS syndrome. […] The outlook for people with POEMS syndrome has improved greatly over the years.

• #70 Characterizing POEMS Syndrome with 18F-FDG PET/CT | Journal of Nuclear Medicine

  https://jnm.snmjournals.org/content/56/9/1334

  POEMS (polyneuropathy, organomegaly, endocrinopathy, M protein elevation, and skin changes) syndrome is a rare paraneoplastic syndrome caused by an underlying plasma cell disorder. The patients usually present with multisystemic involvement. […] The skeletal manifestation on 18F-FDG PET/CT for the patients with POEMS syndrome varied, presenting as both solitary and multiple hypermetabolic bone lesions. […] The total number of 18F-FDGavid bone lesions was 140. […] Four patients showed enlarged and 18F-FDGavid lymph nodes. […] Thirty-six patients had hepatomegaly or splenomegaly. […] Fifty-nine patients showed hypermetabolism of the central BM. […] Serous cavity effusion was commonly seen, especially multiple-cavity effusion. […] Fifteen patients were followed up with PET/CT 3 mo after baseline evaluation and after autologous peripheral blood stem cell transplantation (2 patients received lenalidomide therapy).

• #71 Characterizing POEMS Syndrome with 18F-FDG PET/CT | Journal of Nuclear Medicine

  https://jnm.snmjournals.org/content/56/9/1334

  POEMS (polyneuropathy, organomegaly, endocrinopathy, M protein elevation, and skin changes) syndrome is a rare paraneoplastic syndrome caused by an underlying plasma cell disorder. The patients usually present with multisystemic involvement. […] The skeletal manifestation on 18F-FDG PET/CT for the patients with POEMS syndrome varied, presenting as both solitary and multiple hypermetabolic bone lesions. […] The total number of 18F-FDGavid bone lesions was 140. […] Four patients showed enlarged and 18F-FDGavid lymph nodes. […] Thirty-six patients had hepatomegaly or splenomegaly. […] Fifty-nine patients showed hypermetabolism of the central BM. […] Serous cavity effusion was commonly seen, especially multiple-cavity effusion. […] Fifteen patients were followed up with PET/CT 3 mo after baseline evaluation and after autologous peripheral blood stem cell transplantation (2 patients received lenalidomide therapy).

• #72 POEMS syndrome – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/poems-syndrome/symptoms-causes/syc-20352678

  Not all the symptoms of POEMS syndrome are in the name. And not everyone with POEMS syndrome has all the symptoms in the name. But everyone with POEMS syndrome has polyneuropathy and monoclonal protein. […] Other symptoms some people with POEMS syndrome have spell out PEST. PEST stands for: Papilledema. This is swelling in the back of the eyes. Extravascular volume overload. This might include ankle swelling, fluid between the lung and the ribs, or fluid in the belly. Sclerotic bone lesions. X-rays can show these bone spots. They’re most often not painful. Thrombocytosis and erythrocytosis. These are higher levels of red cells and platelets in the bloodstream. […] Make an appointment with your healthcare professional if you have any of the symptoms of POEMS syndrome. […] The outlook for people with POEMS syndrome has improved greatly over the years.

• #73 POEMS Syndrome Patient Goes from Immobility to Recovery | Dana-Farber Cancer Institute

  https://blog.dana-farber.org/insight/2017/10/poems-syndrome-patient-goes-immobility-recovery/

  A month after his son’s birth, Anyanwu showed the first symptoms of a disease that would soon leave him bedridden, unable to move his legs because of muscle weakness and in unremitting pain in his arms and hands. […] Neuropathy, one of the hallmarks of POEMS syndrome, is a nerve disorder that can produce extreme muscle weakness, pain, numbness, and lack of coordination. […] The imaging tests showed sclerotic lesions areas of abnormal bone in Anyanwu’s pelvis, spine, and ribs. […] Together, these results pointed to POEMS syndrome with demyelinating polyneuropathy the erosion of the protective fatty sheath around the nerves and loss of nerve fibers. […] Shortly after Thanksgiving 2015, Anyanwu had 10 radiation therapy sessions to treat the lesions in his bones. […] I was virtually paralyzed from my legs to my neck, and in severe pain.

• #74 POEMS Syndrome: Background, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/1097031-overview

  The prognosis depends on the extent of the underlying plasma cell disorder and its response to treatment. The prognosis is best for patients with a single lytic lesion, worst for those with a plasma cell disorder involving the bone marrow, and intermediate for those with multiple lytic bone lesions. When the plasma cell disorder responds to treatment, all other symptoms usually improve or resolve completely. […] The natural course of POEMS syndrome is chronic, with a reported median survival of approximately a decade (8-13.8 y). […] Central and peripheral nervous system involvement can lead to significant morbidity and mortality. […] The neurologic sequelae of POEMS syndrome cause approximately 50% of patients with POEMS syndrome to become bedridden.

• #75 POEMS syndrome – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/poems-syndrome/symptoms-causes/syc-20352678

  Not all the symptoms of POEMS syndrome are in the name. And not everyone with POEMS syndrome has all the symptoms in the name. But everyone with POEMS syndrome has polyneuropathy and monoclonal protein. […] Other symptoms some people with POEMS syndrome have spell out PEST. PEST stands for: Papilledema. This is swelling in the back of the eyes. Extravascular volume overload. This might include ankle swelling, fluid between the lung and the ribs, or fluid in the belly. Sclerotic bone lesions. X-rays can show these bone spots. They’re most often not painful. Thrombocytosis and erythrocytosis. These are higher levels of red cells and platelets in the bloodstream. […] Make an appointment with your healthcare professional if you have any of the symptoms of POEMS syndrome. […] The outlook for people with POEMS syndrome has improved greatly over the years.

• #76 POEMS syndrome – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/poems-syndrome/symptoms-causes/syc-20352678

  Not all the symptoms of POEMS syndrome are in the name. And not everyone with POEMS syndrome has all the symptoms in the name. But everyone with POEMS syndrome has polyneuropathy and monoclonal protein. […] Other symptoms some people with POEMS syndrome have spell out PEST. PEST stands for: Papilledema. This is swelling in the back of the eyes. Extravascular volume overload. This might include ankle swelling, fluid between the lung and the ribs, or fluid in the belly. Sclerotic bone lesions. X-rays can show these bone spots. They’re most often not painful. Thrombocytosis and erythrocytosis. These are higher levels of red cells and platelets in the bloodstream. […] Make an appointment with your healthcare professional if you have any of the symptoms of POEMS syndrome. […] The outlook for people with POEMS syndrome has improved greatly over the years.

• #77 POEMS syndrome – Autoimmune Association

  https://autoimmune.org/disease-information/poems-syndrome/

  POEMS syndrome can be misdiagnosed because the signs and symptoms mimic those of other disorders. POEMS syndrome progresses rapidly and may become life-threatening, so early diagnosis is important. […] Signs and symptoms may include progressive sensorimotor polyneuropathy; enlarged liver, spleen, and/or lymph nodes; a disorder of the endocrine glands (often with multiple abnormalities); a monoclonal plasma cell proliferative disorder; and darkening of the skin (hyperpigmentation). Various other symptoms may also occur and may vary among affected people. POEMS syndrome is a chronic disorder, with a median survival time of 8-14 years.

• #78 POEMS Syndrome-Clinical Picture and Management

  https://austinpublishinggroup.com/anatomy/fulltext/Anatomy-v9-id1108.php

  Although probably not a driving force in POEMS, VEGF is considered the best prognostic marker. […] The growth of VEGF correlates best with the activity of the disease. […] A wide range of abnormalities are considered POEMS syndrome connected: hyperpigmentation, hypertrichosis, glomeruloid hemangiomata, plethora, acrocyanosis, flushing, clubbing, white nails. […] Peripheral oedemas are most characteristic, but fluid might accumulate in peritoneum, pleura and pericardium. […] Elevated cytokine production leads to increased vascular permeability and optic disc swelling. […] Hepatomegaly as well as splenomegaly might be present. […] Increased number of platelets and/or hemoglobin level is described as a common laboratory feature in POEMS syndrome. […] In more than 90% patients creatinine level is within normal limits and significant proteinuria is present in less than 10% cases.

• #79 The unclear role of VEGF in POEMS syndrome: therapeutic implications of neoangiogenesis in a rare plasma cell disorder

  https://www.oaepublish.com/articles/2394-4722.2021.106

  POEMS syndrome is a rare paraneoplastic disorder due to an underlying clone of aberrant plasma cells. The clinical presentation can be various and could lead to delayed diagnosis and treatment. Little is known about the pathogenic mechanism, although the neoangiogenesis due to overproduction of vascular endothelial growth factor (VEGF) by plasma cells seems to play a key role. The latest evidence suggests that the blood concentration of this cytokine correlates with the activity of the syndrome: VEGF could then be used as a therapeutic target and a marker to monitor response. […] Even if the role of VEGF in the pathogenesis of POEMS remains unclear, it is still the most important cytokine correlating with disease activity. […] The evidence of high levels of VEGF in serum and plasma of patients with POEMS guided the idea that the syndrome could be successfully treated with drugs directed against this cytokine. […] POEMS syndrome is a rare paraneoplastic disease with a multiorgan involvement which could result fatal in some cases. The mechanism leading to this syndrome is not known, but a central role seems to be played by VEGF.

• #80 POEMS syndrome – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/poems-syndrome/symptoms-causes/syc-20352678

  POEMS syndrome is a rare blood disorder that damages nerves and affects other parts of the body. Symptoms may include weak legs, trouble breathing, a larger spleen or liver, and skin changes. […] Without treatment, POEMS syndrome gets worse quickly. It can become life-threatening. So early diagnosis is vital. […] The symptoms of POEMS syndrome make up its name. Symptoms most often appear over weeks to months. They include the following: Polyneuropathy. This may include numbness, tingling and weakness in the legs. In time, it might affect the hands. It also involves trouble breathing. Polyneuropathy must be present for a POEMS diagnosis. Organomegaly. This is an enlarged spleen, liver or lymph nodes. Endocrinopathy and edema. Atypical hormone levels can cause an underactive thyroid, also called hypothyroidism. The hormone levels also can cause diabetes, sexual issues, tiredness, and swelling in arms and legs. Monoclonal protein. This is a protein that atypical plasma cells in bone marrow make. The monoclonal protein goes into the bloodstream. This must be present for the diagnosis of POEMS syndrome. Monoclonal protein often is linked with bones getting harder or thicker. Skin changes. This may involve having more color than usual on the skin. There may be red spots, which may be harder to see on Black or brown skin. The skin might be thicker. There might be more hair on the face or the legs.

• #81 POEMS Syndrome: What It Is, Symptoms, Causes, and Treatment

  https://www.verywellhealth.com/poems-syndrome-7558580

  POEMS syndrome is expected to progress rapidly and can be life-threatening without treatment. Diagnosis and treatment can help your outcome. […] Without treatment, POEMS syndrome is expected to progress and cause more harm throughout the body. Damage to organs that are already affected may worsen, and additional areas of the body can begin to become involved as well. […] Eventually, without treatment, these effects can become life-threatening.

• #82 POEMS Syndrome: What It Is, Symptoms, Causes, and Treatment

  https://www.verywellhealth.com/poems-syndrome-7558580

  Symptoms can vary, potentially involving abdominal swelling, shortness of breath, and pain. […] The diverse symptoms of POEMS syndrome can develop at any time during the disease course, and they dont have to follow a specific sequence. You might have some symptoms but not others. […] Symptoms of POEMS can include: Swelling in arms and legs: Monoclonal protein accumulation, plasma cell excess, endocrine disorders, or organ failure may cause swelling of the arms and legs. Unexplained weight loss: Organ failure or endocrine disease can affect weight. Bone and joint pain: Hardened areas of the bone can lead to pain and an increased risk of fractures. Vision problems: Papilledema (swelling in the eyes) can cause vision loss. Trouble breathing: Nerve disease can impair the muscles that control breathing. Fatigue: Many of the effects of POEMS syndrome can cause fatigue. A few effects that may contribute to fatigue include thyroid disease, shortness of breath, and swelling. Fever: Sometimes, elevated proteins or plasma cells in the blood can cause a fever.

• #83

  https://haematologica.org/article/view/6589

  The median time from onset to diagnosis in the PH group was longer than that in the non-PH group (26 vs. 14 months, P=0.004). Dyspnea was reported more frequently in patients with PH (50% vs. 19%, P=0.001). Patients with PH were more likely to have ascites (71% vs. 51%, P=0.02), edema (98% vs. 87%, P=0.04), and pleural effusion (62% vs. 37%, P=0.005). […] The incidence of restrictive abnormality (83% vs. 50%, P=0.007) and decreased DLCO (96% vs. 72%, P=0.04) was higher in patients with PH than in those without PH. […] In our study, the PH response rate was 88% in 33 patients at a median eight months after treatment. […] Although a high rate of PH response to treatments was observed in this study, we found that patients with PH had worse median survival than those without (54 months vs. not reached, P=0.021).

• #84 Endocrine Manifestations in POEMS Syndrome: a case report and literature review | BMC Endocrine Disorders | Full Text

  https://bmcendocrdisord.biomedcentral.com/articles/10.1186/s12902-019-0355-6

  The second most common abnormality was adrenal insufficiency, followed by hypothyroidism and subclinical hypothyroidism, then diabetes or glucose intolerance. Additionally, 27-45% of patients suffered from hypocalcemia. More than half had multiple axes involvement, including gonadal, thyroid, pancreas, and adrenal disorders. […] POEMS syndrome is a multisystem disease with a wide spectrum of clinical manifestations and progressive features, which makes its diagnosis a challenge and easily leads to misdiagnosis.

• #85 POEMS Syndrome: What It Is, Symptoms, Causes, and Treatment

  https://www.verywellhealth.com/poems-syndrome-7558580

  POEMS syndrome is expected to progress rapidly and can be life-threatening without treatment. Diagnosis and treatment can help your outcome. […] Without treatment, POEMS syndrome is expected to progress and cause more harm throughout the body. Damage to organs that are already affected may worsen, and additional areas of the body can begin to become involved as well. […] Eventually, without treatment, these effects can become life-threatening.

• #86 Comprehensive Diagnosis and Management of POEMS Syndrome

  https://pmc.ncbi.nlm.nih.gov/articles/PMC9624442/

  Polyneuropathy Organomegaly, Endocrinopathy, Monoclonal protein and Skin changes syndrome is a rare multisystem condition with a range of manifestations which are often overlooked as trivial comorbidities, until their whole triggers the possibility of the diagnosis. The diagnosis is typically delayed by 1216 months, by which time patients can be severely disabled. […] Patients present most often to neurologists or hematologists with progressive peripheral neuropathy and evidence a of monoclonal plasma cell disorder. There are a range of systemic manifestations which are noted but often overlooked as trivial comorbidities. The diagnosis is typically delayed by 1216 months, by which time patients can be severely disabled and bed or wheelchair-bound with established neuropathy. […] Treatment, in the main, improves functionality and leads to encouraging rates of progression-free survival. Furthermore dramatic improvements in overall survival have been achieved in the last 20 years.

• #87 Treatment and outcomes of POEMS syndrome: changes in the past 20 years | Blood Cancer Journal

  https://www.nature.com/articles/s41408-021-00540-1

  Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome is a rare plasma cell dyscrasia with multiorgan involvement. […] The time interval from symptom onset to diagnosis was 23.0 in the entire cohort and 15.9, 12.6, and 12.2 months in the three cohorts. […] Neuropathy was less severe in 2015-2019 than in the other periods, and 35.8, 41.7, and 29.6% of the patients in each group were evaluated as having Overall Neuropathy Limitations Scale (ONLS)4. […] The incidence of hepatomegaly and splenomegaly significantly decreased over time, as well as symptoms related to extravascular volume overload, such as peripheral edema and pleural effusion. […] The incidence of skin changes, such as angioma and hypertrichosis, increased, which may be attributed to the increased understanding of the disease and more careful physical evaluation. […] The 5-year OS rates in the three periods were 70.0, 85.4, and 92.2%. […] Overall survival increased significantly, mainly due to dramatic advances in non-ASCT therapeutic options.

• #88 Comprehensive Diagnosis and Management of POEMS Syndrome

  https://pmc.ncbi.nlm.nih.gov/articles/PMC9624442/

  Polyneuropathy Organomegaly, Endocrinopathy, Monoclonal protein and Skin changes syndrome is a rare multisystem condition with a range of manifestations which are often overlooked as trivial comorbidities, until their whole triggers the possibility of the diagnosis. The diagnosis is typically delayed by 1216 months, by which time patients can be severely disabled. […] Patients present most often to neurologists or hematologists with progressive peripheral neuropathy and evidence a of monoclonal plasma cell disorder. There are a range of systemic manifestations which are noted but often overlooked as trivial comorbidities. The diagnosis is typically delayed by 1216 months, by which time patients can be severely disabled and bed or wheelchair-bound with established neuropathy. […] Treatment, in the main, improves functionality and leads to encouraging rates of progression-free survival. Furthermore dramatic improvements in overall survival have been achieved in the last 20 years.

• #89 Orphanet: POEMS syndrome

  https://www.orpha.net/en/disease/detail/2905

  POEMS syndrome is a paraneoplastic syndrome characterized by polyradiculoneuropathy (P), organomegaly (O), endocrinopathy (E), clonal plasma cell disorder (M), and skin changes (S). […] Neuropathy is the primary manifestation and is usually peripheral, ascending, and symmetrical with distal weakness and paresthesia. It is both sensory and motor and may cause pain, hyperesthesia, and areflexia. A clonal plasma cell disorder (PCD) is the second major feature. Organomegaly mainly includes hepatomegaly, splenomegaly, and/or lymphadenopathy (Castleman disease). Various and extensive endocrine disorders may be found causing hypogonadism, thyroid abnormalities, adrenal insufficiency, and/or erectile dysfunction and gynecomastia in men. The skin is commonly affected with development of hyperpigmentation, hemangiomas, hypertrichosis, leukonychia or nail clubbing, sclerodermoid changes, lipodystrophy, and flushing. The main ocular manifestation is papilledema. Extravascular overload mainly manifests as peripheral edema, and sometimes as pleural effusion, ascites, and pericardial effusions. An increased risk of arterial and/or venous thrombosis is reported (up to 20% of patients). Disease severity depends on the systems involved and the course is chronic. Some patients have a mild presentation while others have debilitating disease. 25% have respiratory manifestations including restrictive lung disease, pulmonary hypertension, and respiratory muscle weakness. […] The prognosis is generally good if diagnosis is early and appropriate treatment is provided. Causes of death include progressive anasarca, thrombosis, and inanition.

• #90 POEMS syndrome | MedLink Neurology

  https://www.medlink.com/articles/poems-syndrome

  Endocrine abnormalities vary among reported series, usually due to hormone insufficiency involving the four major endocrine axes (gonadal, thyroid, glucose metabolism, and adrenal). In one large series from the Mayo Clinic, 84% of patients had a recognized endocrinopathy, hypogonadism being the most common. […] In a large series of 137 patients with POEMS syndrome from the Mayo Clinic, 28% presented with pulmonary manifestations, including pulmonary hypertension, restrictive lung disease, respiratory muscle weakness, and an isolated diminished diffusing capacity. […] In the first published series of patients with POEMS syndrome, a mean survival of 33 months and an estimated 5-year survival of 60% were reported. In a subsequent large series of 291 patients from the Mayo Clinic, 83 of whom (28.5%) had received an autologous transplant, a 10-year overall survival of 62% was reported.

• #91 POEMS Syndrome-Clinical Picture and Management

  https://austinpublishinggroup.com/anatomy/fulltext/Anatomy-v9-id1108.php

  Pulmonary involvement may vary in severity in POEMS: restrictive lung disease, pulmonary hypertension, reduced diffusion capacity of carbon monoxide. […] Based on small studies arterial and venous thrombosis is claimed to appear in 20% of POEMS patients. […] The number of clinical features present at diagnosis does not determine survival in POEMS. […] Extravascular volume overload, pulmonary disorders and ischemic complications are associated with unfavorable prognosis in POEMS. […] Prognostic factors of potential value are listed in Table 3. […] Neurologic response is usually seen no sooner than after 6 months after treatment implementation, one has to wait for 2-3 years for maximal effect. […] Although clinical response means quality of life improvement, it is hematological remission that influences patients outcome.

• #92 POEMS Syndrome-Clinical Picture and Management

  https://austinpublishinggroup.com/anatomy/fulltext/Anatomy-v9-id1108.php

  Pulmonary involvement may vary in severity in POEMS: restrictive lung disease, pulmonary hypertension, reduced diffusion capacity of carbon monoxide. […] Based on small studies arterial and venous thrombosis is claimed to appear in 20% of POEMS patients. […] The number of clinical features present at diagnosis does not determine survival in POEMS. […] Extravascular volume overload, pulmonary disorders and ischemic complications are associated with unfavorable prognosis in POEMS. […] Prognostic factors of potential value are listed in Table 3. […] Neurologic response is usually seen no sooner than after 6 months after treatment implementation, one has to wait for 2-3 years for maximal effect. […] Although clinical response means quality of life improvement, it is hematological remission that influences patients outcome.

• #93

  https://haematologica.org/article/view/6589

  POEMS syndrome is a rare clonal plasma cell disease. Patients with POEMS syndrome are at risk of developing pulmonary hypertension, but the data on its incidence and impact on outcome are limited. […] Forty-two (27%) of 154 patients with pulmonary hypertension (estimated sPAP 50mmHg) were identified. […] Patients with pulmonary hypertension were more likely to have peripheral edema (P=0.04), ascites (P=0.02), pleural effusion (P=0.005), and have longer time from onset to diagnosis (P=0.004) when compared with those without pulmonary hypertension. […] Reversibility of pulmonary hypertension was observed after treatment of POEMS syndrome. After median follow of 32 months, survival of patients with pulmonary hypertension was worse than those without (median overall survival 54 months vs. median not reached, P=0.021). In conclusion, pulmonary hypertension is a common feature of POEMS syndrome, and is associated with signs of extravascular volume overload. Although active treatment of POEMS syndrome can reverse pulmonary hypertension, survival of these patients is worse than those without pulmonary hypertension.

• #94

  https://haematologica.org/article/view/6589

  The median time from onset to diagnosis in the PH group was longer than that in the non-PH group (26 vs. 14 months, P=0.004). Dyspnea was reported more frequently in patients with PH (50% vs. 19%, P=0.001). Patients with PH were more likely to have ascites (71% vs. 51%, P=0.02), edema (98% vs. 87%, P=0.04), and pleural effusion (62% vs. 37%, P=0.005). […] The incidence of restrictive abnormality (83% vs. 50%, P=0.007) and decreased DLCO (96% vs. 72%, P=0.04) was higher in patients with PH than in those without PH. […] In our study, the PH response rate was 88% in 33 patients at a median eight months after treatment. […] Although a high rate of PH response to treatments was observed in this study, we found that patients with PH had worse median survival than those without (54 months vs. not reached, P=0.021).

• #95 POEMS Syndrome | Condition | UAMS Health

  https://uamshealth.com/condition/poems-syndrome/

  Additional symptoms may include fluid buildup in the lungs, chronic renal failure, fluid accumulation in the skin of the arms and legs, and cardiovascular disease. […] Patients with POEMS syndrome often need treatment to alleviate the symptoms of neuropathy, extravascular volume overload, and endocrine abnormalities.

• #96 Oncology Letters

  https://www.spandidos-publications.com/10.3892/ol.2017.6904

  Weakness in the upper and lower limbs is a common symptom of multiple diseases, including neuromuscular disorders, amyotrophic lateral sclerosis, multiple sclerosis, Guillain-Barre syndrome, transverse myelitis, hypovitaminosis D and POEMS syndrome. Hence, presentation with weakness in the upper and lower limbs in patients with POEMS syndrome may not result in the correct diagnosis. The patient with POEMS syndrome described in the present study initially presented with weakness in the upper and lower limbs 2 years ago, and subsequently succumbed to respiratory failure at age 50 due to delayed diagnosis of POEMS syndrome. […] Respiratory failure induced mortality occurred 24 months after the patient first experienced difficulty walking and numbness in her lower extremities. The present study suggests that abnormal symptoms in cases of POEMS syndrome should be further evaluated during the diagnosis and treatment.

• #97 A Review of POEMS Syndrome

  https://www.cancernetwork.com/view/review-poems-syndrome

  Patients with POEMS syndrome are at increased risk for arterial and/or venous thromboses during the course of their disease, with nearly 20% of patients experiencing one of these complications. […] The course of POEMS syndrome is usually chronic, with a median patient survival time of nearly 14 years. […] Only fingernail clubbing, extravascular volume overload (effusions, edema, and ascites), and respiratory symptoms have been associated with a significantly shorter overall survival time. […] The number of POEMS features does not affect survival. […] Once there is disseminated bone marrow involvement, albeit even in patients with a low plasma cell percentage, radiation is not expected to be curative. […] It is imperative that the treating physician (and patient) realize that there is a lag between completion of successful therapy and neurologic response, often with no discernible improvement until 3 to 6 months after completion of therapy. Maximal response is not seen until 2 to 3 years afterwards. Other features, such as anasarca, papilledema, and even skin changes, typically improve sooner.

• #98 POEMS Syndrome-Clinical Picture and Management

  https://austinpublishinggroup.com/anatomy/fulltext/Anatomy-v9-id1108.php

  Pulmonary involvement may vary in severity in POEMS: restrictive lung disease, pulmonary hypertension, reduced diffusion capacity of carbon monoxide. […] Based on small studies arterial and venous thrombosis is claimed to appear in 20% of POEMS patients. […] The number of clinical features present at diagnosis does not determine survival in POEMS. […] Extravascular volume overload, pulmonary disorders and ischemic complications are associated with unfavorable prognosis in POEMS. […] Prognostic factors of potential value are listed in Table 3. […] Neurologic response is usually seen no sooner than after 6 months after treatment implementation, one has to wait for 2-3 years for maximal effect. […] Although clinical response means quality of life improvement, it is hematological remission that influences patients outcome.

• #99 POEMS Syndrome | Condition | UAMS Health

  https://uamshealth.com/condition/poems-syndrome/

  Additional symptoms may include fluid buildup in the lungs, chronic renal failure, fluid accumulation in the skin of the arms and legs, and cardiovascular disease. […] Patients with POEMS syndrome often need treatment to alleviate the symptoms of neuropathy, extravascular volume overload, and endocrine abnormalities.

• #100 POEMS Syndrome-Clinical Picture and Management

  https://austinpublishinggroup.com/anatomy/fulltext/Anatomy-v9-id1108.php

  Pulmonary involvement may vary in severity in POEMS: restrictive lung disease, pulmonary hypertension, reduced diffusion capacity of carbon monoxide. […] Based on small studies arterial and venous thrombosis is claimed to appear in 20% of POEMS patients. […] The number of clinical features present at diagnosis does not determine survival in POEMS. […] Extravascular volume overload, pulmonary disorders and ischemic complications are associated with unfavorable prognosis in POEMS. […] Prognostic factors of potential value are listed in Table 3. […] Neurologic response is usually seen no sooner than after 6 months after treatment implementation, one has to wait for 2-3 years for maximal effect. […] Although clinical response means quality of life improvement, it is hematological remission that influences patients outcome.

• #101 An Atypical Case of POEMS Syndrome Associated with Autonomic Dysfunction

  https://openneurologyjournal.com/VOLUME/13/PAGE/92/FULLTEXT/

  A 52 year old patient showed, for two years, symptoms compatible with sensorimotor polyneuropathy preventing him from walking. […] The patient did not respond to corticosteroid therapy and later suffered sexual Dysfunction, Swelling, Lymphadenopathy, Hypotension, astrointestinal dysmotility, urinary retention and neuropathic pain. […] The most striking feature, in this case, was the patients heightened and atypical polyneuropathy without axonal injury even after an extended period of time, and significant and atypical dysautonomia. […] Autonomic function preservation is typical of this syndrome and distinguishes it from other neuropathies such as diabetic neuropathy. […] During hospitalization, he suffered respiratory failure, requiring orotracheal intubation, dysautonomia with refractory arterial hypotension, xerostomia, urinary retention and constipation.

• #102 An Atypical Case of POEMS Syndrome Associated with Autonomic Dysfunction

  https://openneurologyjournal.com/VOLUME/13/PAGE/92/

  The patient also showed dysautonomia signs that began with sexual dysfunction and progressed to heat intolerance, severe arterial hypotension, tachycardia, urinary retention, chronic constipation and xerostomia. […] The autonomic dysfunction pattern is unusual for POEMS syndrome and suggests widening diagnostic possibilities in the field of demyelinating polyneuropathy. […] However occasionally, atypical cases without axonal lesions and significant autonomic dysfunction are observed.

• #103 An Atypical Case of POEMS Syndrome Associated with Autonomic Dysfunction

  https://openneurologyjournal.com/VOLUME/13/PAGE/92/

  A 52 year old patient showed, for two years, symptoms compatible with sensorimotor polyneuropathy preventing him from walking. […] The patient did not respond to corticosteroid therapy and later suffered sexual Dysfunction, Swelling, Lymphadenopathy, Hypotension, astrointestinal dysmotility, urinary retention and neuropathic pain. […] The most striking feature, in this case, was the patients heightened and atypical polyneuropathy without axonal injury even after an extended period of time, and significant and atypical dysautonomia. […] POEMS syndromes clinical spectrum is fairly extensive. Peripheral nerves are a major target and the disorder is usually found in polyneuropathy patients with preserved autonomic function despite severe motor dysfunction. […] This paper reports the case of a POEMS syndrome patient who critically showed signs of dysautonomia, which strengthens the need for research on atypical cases of this disorder.

• #104 An Atypical Case of POEMS Syndrome Associated with Autonomic Dysfunction

  https://openneurologyjournal.com/VOLUME/13/PAGE/92/FULLTEXT/

  The patient also showed dysautonomia signs that began with sexual dysfunction and progressed to heat intolerance, severe arterial hypotension, tachycardia, urinary retention, chronic constipation and xerostomia. […] Although the reported patients clinical conditions were consistent with the syndrome, his neuropathy pattern was unusual. […] This report is significant as this atypical presentation draws attention to the syndromes diagnosis.

• #105 An Atypical Case of POEMS Syndrome Associated with Autonomic Dysfunction

  https://openneurologyjournal.com/VOLUME/13/PAGE/92/FULLTEXT/

  The patient also showed dysautonomia signs that began with sexual dysfunction and progressed to heat intolerance, severe arterial hypotension, tachycardia, urinary retention, chronic constipation and xerostomia. […] Although the reported patients clinical conditions were consistent with the syndrome, his neuropathy pattern was unusual. […] This report is significant as this atypical presentation draws attention to the syndromes diagnosis.

• #106 An Atypical Case of POEMS Syndrome Associated with Autonomic Dysfunction

  https://openneurologyjournal.com/VOLUME/13/PAGE/92/FULLTEXT/

  The patient also showed dysautonomia signs that began with sexual dysfunction and progressed to heat intolerance, severe arterial hypotension, tachycardia, urinary retention, chronic constipation and xerostomia. […] Although the reported patients clinical conditions were consistent with the syndrome, his neuropathy pattern was unusual. […] This report is significant as this atypical presentation draws attention to the syndromes diagnosis.

• #107 An Atypical Case of POEMS Syndrome Associated with Autonomic Dysfunction

  https://openneurologyjournal.com/VOLUME/13/PAGE/92/FULLTEXT/

  A 52 year old patient showed, for two years, symptoms compatible with sensorimotor polyneuropathy preventing him from walking. […] The patient did not respond to corticosteroid therapy and later suffered sexual Dysfunction, Swelling, Lymphadenopathy, Hypotension, astrointestinal dysmotility, urinary retention and neuropathic pain. […] The most striking feature, in this case, was the patients heightened and atypical polyneuropathy without axonal injury even after an extended period of time, and significant and atypical dysautonomia. […] Autonomic function preservation is typical of this syndrome and distinguishes it from other neuropathies such as diabetic neuropathy. […] During hospitalization, he suffered respiratory failure, requiring orotracheal intubation, dysautonomia with refractory arterial hypotension, xerostomia, urinary retention and constipation.

• #108 An Atypical Case of POEMS Syndrome Associated with Autonomic Dysfunction

  https://openneurologyjournal.com/VOLUME/13/PAGE/92/FULLTEXT/

  The patient also showed dysautonomia signs that began with sexual dysfunction and progressed to heat intolerance, severe arterial hypotension, tachycardia, urinary retention, chronic constipation and xerostomia. […] Although the reported patients clinical conditions were consistent with the syndrome, his neuropathy pattern was unusual. […] This report is significant as this atypical presentation draws attention to the syndromes diagnosis.

• #109 An Atypical Case of POEMS Syndrome Associated with Autonomic Dysfunction

  https://openneurologyjournal.com/VOLUME/13/PAGE/92/FULLTEXT/

  The patient also showed dysautonomia signs that began with sexual dysfunction and progressed to heat intolerance, severe arterial hypotension, tachycardia, urinary retention, chronic constipation and xerostomia. […] Although the reported patients clinical conditions were consistent with the syndrome, his neuropathy pattern was unusual. […] This report is significant as this atypical presentation draws attention to the syndromes diagnosis.

• #110 POEMS syndrome – Autoimmune Association

  https://autoimmune.org/disease-information/poems-syndrome/

  POEMS syndrome can be misdiagnosed because the signs and symptoms mimic those of other disorders. POEMS syndrome progresses rapidly and may become life-threatening, so early diagnosis is important. […] Signs and symptoms may include progressive sensorimotor polyneuropathy; enlarged liver, spleen, and/or lymph nodes; a disorder of the endocrine glands (often with multiple abnormalities); a monoclonal plasma cell proliferative disorder; and darkening of the skin (hyperpigmentation). Various other symptoms may also occur and may vary among affected people. POEMS syndrome is a chronic disorder, with a median survival time of 8-14 years.

• #111 POEMS Syndrome: Background, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/1097031-overview

  The prognosis depends on the extent of the underlying plasma cell disorder and its response to treatment. The prognosis is best for patients with a single lytic lesion, worst for those with a plasma cell disorder involving the bone marrow, and intermediate for those with multiple lytic bone lesions. When the plasma cell disorder responds to treatment, all other symptoms usually improve or resolve completely. […] The natural course of POEMS syndrome is chronic, with a reported median survival of approximately a decade (8-13.8 y). […] Central and peripheral nervous system involvement can lead to significant morbidity and mortality. […] The neurologic sequelae of POEMS syndrome cause approximately 50% of patients with POEMS syndrome to become bedridden.

• #112 What Is POEMS Syndrome – Klarity Health Library

  https://my.klarity.health/what-is-poems-syndrome/

  The main goal of treatment is to slow the growth of plasma cell neoplasms and the release of pro-inflammatory and pro-angiogenic cytokines. As soon as a diagnosis is confirmed, you should expect to start treatment right away. Your condition can progressively worsen if you don’t receive any treatment. […] The prognosis for POEMS syndrome is good in comparison with multiple myeloma. The management and therapies for POEMS syndrome have greatly improved since the 1980s, when the median survival was 33 months. Today, approximately 82% of newly diagnosed patients can expect to live for more than 10 years. […] Patients with nail clubbing or peripheral fluid overload after treatment have shorter survival expectations, approximately 31 months and 79 months respectively.

• #113 POEMS Syndrome : Life expectancy, Treatment, Symptoms, Causes

  https://anavara.com/treatment/poems-syndrome

  Plasma cells dyscrasias; produce a monoclonal protein that can be found in the bloodstream. Hardening and thickening of bones. […] Hyperpigmentation (dark-colored skin). Skin becomes thicker (scleroderma). Red-spotted skin. Excessive sweating. Clubbed fingernails. More facial and leg hair. […] Visual disturbance, headache, and nausea. Pericardial effusion. Fluid in arms and legs. Weight loss. Fatigue. […] POEMS syndrome is a chronic disorder with a reported median survival of approximately 10 years and a reported 5-year survival rate of 60%. […] One should not misconstrue the signs and symptoms of POEMS syndrome for other disorders such as osteoporosis and arthritis. Early diagnosis is important because POEMS syndrome advancement can be very life-threatening.

• #114 :: JKMS :: Journal of Korean Medical Science

  https://jkms.org/DOIx.php?id=10.3346/jkms.2024.39.e5

  POEMS syndrome is a rare form of plasma cell dyscrasia characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal proteins, and skin changes. […] All patients had peripheral neuropathy, and 81 (96.4%) had monoclonal plasma cell proliferation. Other common features included skin changes (54.2%), volume overload (71.4%), and organomegaly (72.6%). […] The median follow-up duration was 40.7 months. The 5-year overall survival (OS) was 78%, and the 5-year progression-free survival (PFS) was 55%. Patients who underwent upfront ASCT and were diagnosed after 2014 had a longer OS and PFS. […] The median time from neurological symptom presentation to the diagnosis of POEMS syndrome was 173 days (range, 0-3969 days). […] Elevated plasma VEGF levels were significantly associated with the presence of ascites and overall volume overload but not with peripheral edema, pleural effusion, pericardial effusion, or pulmonary hypertension.

• #115 POEMS syndrome | MedLink Neurology

  https://www.medlink.com/articles/poems-syndrome

  Endocrine abnormalities vary among reported series, usually due to hormone insufficiency involving the four major endocrine axes (gonadal, thyroid, glucose metabolism, and adrenal). In one large series from the Mayo Clinic, 84% of patients had a recognized endocrinopathy, hypogonadism being the most common. […] In a large series of 137 patients with POEMS syndrome from the Mayo Clinic, 28% presented with pulmonary manifestations, including pulmonary hypertension, restrictive lung disease, respiratory muscle weakness, and an isolated diminished diffusing capacity. […] In the first published series of patients with POEMS syndrome, a mean survival of 33 months and an estimated 5-year survival of 60% were reported. In a subsequent large series of 291 patients from the Mayo Clinic, 83 of whom (28.5%) had received an autologous transplant, a 10-year overall survival of 62% was reported.

• #116 :: JKMS :: Journal of Korean Medical Science

  https://jkms.org/DOIx.php?id=10.3346/jkms.2024.39.e5

  The CRH rate, CRV rate, and clinical improvement rate were 67.2%, 70.0%, and 82.0%, respectively. The median OS was not reached (5-year OS: 78%; 10-year OS: 65%), and the median PFS was 71.4 months (5-year PFS: 55%; 10-year PFS: 37%). […] The diagnosis of POEMS syndrome required keen observation of patients with related symptoms, with a median duration of 173 days from neurological symptoms to diagnosis, which was shorter than that reported previously. […] The treatment outcomes of Korean patients with POEMS syndrome have improved in recent years.

• #117 A Review of POEMS Syndrome

  https://www.cancernetwork.com/view/review-poems-syndrome

  Patients with POEMS syndrome are at increased risk for arterial and/or venous thromboses during the course of their disease, with nearly 20% of patients experiencing one of these complications. […] The course of POEMS syndrome is usually chronic, with a median patient survival time of nearly 14 years. […] Only fingernail clubbing, extravascular volume overload (effusions, edema, and ascites), and respiratory symptoms have been associated with a significantly shorter overall survival time. […] The number of POEMS features does not affect survival. […] Once there is disseminated bone marrow involvement, albeit even in patients with a low plasma cell percentage, radiation is not expected to be curative. […] It is imperative that the treating physician (and patient) realize that there is a lag between completion of successful therapy and neurologic response, often with no discernible improvement until 3 to 6 months after completion of therapy. Maximal response is not seen until 2 to 3 years afterwards. Other features, such as anasarca, papilledema, and even skin changes, typically improve sooner.

• #118 POEMS Syndrome-Clinical Picture and Management

  https://austinpublishinggroup.com/anatomy/fulltext/Anatomy-v9-id1108.php

  Pulmonary involvement may vary in severity in POEMS: restrictive lung disease, pulmonary hypertension, reduced diffusion capacity of carbon monoxide. […] Based on small studies arterial and venous thrombosis is claimed to appear in 20% of POEMS patients. […] The number of clinical features present at diagnosis does not determine survival in POEMS. […] Extravascular volume overload, pulmonary disorders and ischemic complications are associated with unfavorable prognosis in POEMS. […] Prognostic factors of potential value are listed in Table 3. […] Neurologic response is usually seen no sooner than after 6 months after treatment implementation, one has to wait for 2-3 years for maximal effect. […] Although clinical response means quality of life improvement, it is hematological remission that influences patients outcome.

• #119 What Is POEMS Syndrome – Klarity Health Library

  https://my.klarity.health/what-is-poems-syndrome/

  The main goal of treatment is to slow the growth of plasma cell neoplasms and the release of pro-inflammatory and pro-angiogenic cytokines. As soon as a diagnosis is confirmed, you should expect to start treatment right away. Your condition can progressively worsen if you don’t receive any treatment. […] The prognosis for POEMS syndrome is good in comparison with multiple myeloma. The management and therapies for POEMS syndrome have greatly improved since the 1980s, when the median survival was 33 months. Today, approximately 82% of newly diagnosed patients can expect to live for more than 10 years. […] Patients with nail clubbing or peripheral fluid overload after treatment have shorter survival expectations, approximately 31 months and 79 months respectively.

• #120 What Is POEMS Syndrome – Klarity Health Library

  https://my.klarity.health/what-is-poems-syndrome/

  The main goal of treatment is to slow the growth of plasma cell neoplasms and the release of pro-inflammatory and pro-angiogenic cytokines. As soon as a diagnosis is confirmed, you should expect to start treatment right away. Your condition can progressively worsen if you don’t receive any treatment. […] The prognosis for POEMS syndrome is good in comparison with multiple myeloma. The management and therapies for POEMS syndrome have greatly improved since the 1980s, when the median survival was 33 months. Today, approximately 82% of newly diagnosed patients can expect to live for more than 10 years. […] Patients with nail clubbing or peripheral fluid overload after treatment have shorter survival expectations, approximately 31 months and 79 months respectively.

• #121 A Review of POEMS Syndrome

  https://www.cancernetwork.com/view/review-poems-syndrome

  Patients with POEMS syndrome are at increased risk for arterial and/or venous thromboses during the course of their disease, with nearly 20% of patients experiencing one of these complications. […] The course of POEMS syndrome is usually chronic, with a median patient survival time of nearly 14 years. […] Only fingernail clubbing, extravascular volume overload (effusions, edema, and ascites), and respiratory symptoms have been associated with a significantly shorter overall survival time. […] The number of POEMS features does not affect survival. […] Once there is disseminated bone marrow involvement, albeit even in patients with a low plasma cell percentage, radiation is not expected to be curative. […] It is imperative that the treating physician (and patient) realize that there is a lag between completion of successful therapy and neurologic response, often with no discernible improvement until 3 to 6 months after completion of therapy. Maximal response is not seen until 2 to 3 years afterwards. Other features, such as anasarca, papilledema, and even skin changes, typically improve sooner.

• #122

  https://haematologica.org/article/view/6589

  The median time from onset to diagnosis in the PH group was longer than that in the non-PH group (26 vs. 14 months, P=0.004). Dyspnea was reported more frequently in patients with PH (50% vs. 19%, P=0.001). Patients with PH were more likely to have ascites (71% vs. 51%, P=0.02), edema (98% vs. 87%, P=0.04), and pleural effusion (62% vs. 37%, P=0.005). […] The incidence of restrictive abnormality (83% vs. 50%, P=0.007) and decreased DLCO (96% vs. 72%, P=0.04) was higher in patients with PH than in those without PH. […] In our study, the PH response rate was 88% in 33 patients at a median eight months after treatment. […] Although a high rate of PH response to treatments was observed in this study, we found that patients with PH had worse median survival than those without (54 months vs. not reached, P=0.021).

• #123 POEMS Syndrome: 2019 Update on diagnosis, risk-stratification, and management – PubMed

  https://pubmed.ncbi.nlm.nih.gov/31012139/

  Polyneuropathy, organomegaly, endocrinopathy, M-protein, skin changes (POEMS) syndrome is a paraneoplastic syndrome due to an underlying plasma cell neoplasm. The major criteria for the syndrome are polyradiculoneuropathy, clonal plasma cell disorder (PCD), sclerotic bone lesions, elevated vascular endothelial growth factor, and the presence of Castleman disease. Minor features include organomegaly, endocrinopathy, characteristic skin changes, papilledema, extravascular volume overload, and thrombocytosis. […] The diagnosis of POEMS syndrome is made with three of the major criteria, two of which must include polyradiculoneuropathy and clonal PCD, and at least one of the minor criteria. […] Risk factors include low serum albumin, age, pleural effusion, pulmonary hypertension, and reduced eGFR.

• #124 POEMS Syndrome: 2019 Update on diagnosis, risk-stratification, and management – PubMed

  https://pubmed.ncbi.nlm.nih.gov/31012139/

  Polyneuropathy, organomegaly, endocrinopathy, M-protein, skin changes (POEMS) syndrome is a paraneoplastic syndrome due to an underlying plasma cell neoplasm. The major criteria for the syndrome are polyradiculoneuropathy, clonal plasma cell disorder (PCD), sclerotic bone lesions, elevated vascular endothelial growth factor, and the presence of Castleman disease. Minor features include organomegaly, endocrinopathy, characteristic skin changes, papilledema, extravascular volume overload, and thrombocytosis. […] The diagnosis of POEMS syndrome is made with three of the major criteria, two of which must include polyradiculoneuropathy and clonal PCD, and at least one of the minor criteria. […] Risk factors include low serum albumin, age, pleural effusion, pulmonary hypertension, and reduced eGFR.

• #125 POEMS Syndrome: 2019 Update on diagnosis, risk-stratification, and management – PubMed

  https://pubmed.ncbi.nlm.nih.gov/31012139/

  Polyneuropathy, organomegaly, endocrinopathy, M-protein, skin changes (POEMS) syndrome is a paraneoplastic syndrome due to an underlying plasma cell neoplasm. The major criteria for the syndrome are polyradiculoneuropathy, clonal plasma cell disorder (PCD), sclerotic bone lesions, elevated vascular endothelial growth factor, and the presence of Castleman disease. Minor features include organomegaly, endocrinopathy, characteristic skin changes, papilledema, extravascular volume overload, and thrombocytosis. […] The diagnosis of POEMS syndrome is made with three of the major criteria, two of which must include polyradiculoneuropathy and clonal PCD, and at least one of the minor criteria. […] Risk factors include low serum albumin, age, pleural effusion, pulmonary hypertension, and reduced eGFR.

• #126 A Review of POEMS Syndrome

  https://www.cancernetwork.com/view/review-poems-syndrome

  Patients with POEMS syndrome are at increased risk for arterial and/or venous thromboses during the course of their disease, with nearly 20% of patients experiencing one of these complications. […] The course of POEMS syndrome is usually chronic, with a median patient survival time of nearly 14 years. […] Only fingernail clubbing, extravascular volume overload (effusions, edema, and ascites), and respiratory symptoms have been associated with a significantly shorter overall survival time. […] The number of POEMS features does not affect survival. […] Once there is disseminated bone marrow involvement, albeit even in patients with a low plasma cell percentage, radiation is not expected to be curative. […] It is imperative that the treating physician (and patient) realize that there is a lag between completion of successful therapy and neurologic response, often with no discernible improvement until 3 to 6 months after completion of therapy. Maximal response is not seen until 2 to 3 years afterwards. Other features, such as anasarca, papilledema, and even skin changes, typically improve sooner.

• #127 POEMS Syndrome-Clinical Picture and Management

  https://austinpublishinggroup.com/anatomy/fulltext/Anatomy-v9-id1108.php

  Pulmonary involvement may vary in severity in POEMS: restrictive lung disease, pulmonary hypertension, reduced diffusion capacity of carbon monoxide. […] Based on small studies arterial and venous thrombosis is claimed to appear in 20% of POEMS patients. […] The number of clinical features present at diagnosis does not determine survival in POEMS. […] Extravascular volume overload, pulmonary disorders and ischemic complications are associated with unfavorable prognosis in POEMS. […] Prognostic factors of potential value are listed in Table 3. […] Neurologic response is usually seen no sooner than after 6 months after treatment implementation, one has to wait for 2-3 years for maximal effect. […] Although clinical response means quality of life improvement, it is hematological remission that influences patients outcome.

• #128 Multiple Myeloma: POEMS Syndrome | Memorial Sloan Kettering Cancer Center

  https://www.mskcc.org/cancer-care/types/multiple-myeloma/poems-syndrome

  Skin lesions mean changes in the skin, including its color. There can be thickening of your skin, changes in color, and red or purple spots. […] POEMS can get worse quickly. It can be very serious if you do not start treatment right away. […] The treatment goal for POEMS syndrome is to help your symptoms. There is no cure for the disease. But when the disease is controlled, people who have POEMS syndrome will improve. […] Treatments for the symptoms of POEMS are the same as for multiple myeloma.

• #129 A Review of POEMS Syndrome

  https://www.cancernetwork.com/view/review-poems-syndrome

  Patients with POEMS syndrome are at increased risk for arterial and/or venous thromboses during the course of their disease, with nearly 20% of patients experiencing one of these complications. […] The course of POEMS syndrome is usually chronic, with a median patient survival time of nearly 14 years. […] Only fingernail clubbing, extravascular volume overload (effusions, edema, and ascites), and respiratory symptoms have been associated with a significantly shorter overall survival time. […] The number of POEMS features does not affect survival. […] Once there is disseminated bone marrow involvement, albeit even in patients with a low plasma cell percentage, radiation is not expected to be curative. […] It is imperative that the treating physician (and patient) realize that there is a lag between completion of successful therapy and neurologic response, often with no discernible improvement until 3 to 6 months after completion of therapy. Maximal response is not seen until 2 to 3 years afterwards. Other features, such as anasarca, papilledema, and even skin changes, typically improve sooner.

• #130 A Review of POEMS Syndrome

  https://www.cancernetwork.com/view/review-poems-syndrome

  Patients with POEMS syndrome are at increased risk for arterial and/or venous thromboses during the course of their disease, with nearly 20% of patients experiencing one of these complications. […] The course of POEMS syndrome is usually chronic, with a median patient survival time of nearly 14 years. […] Only fingernail clubbing, extravascular volume overload (effusions, edema, and ascites), and respiratory symptoms have been associated with a significantly shorter overall survival time. […] The number of POEMS features does not affect survival. […] Once there is disseminated bone marrow involvement, albeit even in patients with a low plasma cell percentage, radiation is not expected to be curative. […] It is imperative that the treating physician (and patient) realize that there is a lag between completion of successful therapy and neurologic response, often with no discernible improvement until 3 to 6 months after completion of therapy. Maximal response is not seen until 2 to 3 years afterwards. Other features, such as anasarca, papilledema, and even skin changes, typically improve sooner.

• #131 Comprehensive Diagnosis and Management of POEMS Syndrome

  https://pmc.ncbi.nlm.nih.gov/articles/PMC9624442/

  Most patients with POEMS syndrome respond favorably to treatment, but neurological improvement may not commence for 2 years after treatment and may continue for years. Chronic and irreversible functional impairment following treatment is common and requires ongoing monitoring and support. Some is preventable and early provision of occupational and physiotherapy support to maintain passive and active movement, reduce tendon contractures and pain and prevent complications of immobility is invaluable.

• #132 A Review of POEMS Syndrome

  https://www.cancernetwork.com/view/review-poems-syndrome

  Patients with POEMS syndrome are at increased risk for arterial and/or venous thromboses during the course of their disease, with nearly 20% of patients experiencing one of these complications. […] The course of POEMS syndrome is usually chronic, with a median patient survival time of nearly 14 years. […] Only fingernail clubbing, extravascular volume overload (effusions, edema, and ascites), and respiratory symptoms have been associated with a significantly shorter overall survival time. […] The number of POEMS features does not affect survival. […] Once there is disseminated bone marrow involvement, albeit even in patients with a low plasma cell percentage, radiation is not expected to be curative. […] It is imperative that the treating physician (and patient) realize that there is a lag between completion of successful therapy and neurologic response, often with no discernible improvement until 3 to 6 months after completion of therapy. Maximal response is not seen until 2 to 3 years afterwards. Other features, such as anasarca, papilledema, and even skin changes, typically improve sooner.

• #133 POEMS Syndrome-Clinical Picture and Management

  https://austinpublishinggroup.com/anatomy/fulltext/Anatomy-v9-id1108.php

  Pulmonary involvement may vary in severity in POEMS: restrictive lung disease, pulmonary hypertension, reduced diffusion capacity of carbon monoxide. […] Based on small studies arterial and venous thrombosis is claimed to appear in 20% of POEMS patients. […] The number of clinical features present at diagnosis does not determine survival in POEMS. […] Extravascular volume overload, pulmonary disorders and ischemic complications are associated with unfavorable prognosis in POEMS. […] Prognostic factors of potential value are listed in Table 3. […] Neurologic response is usually seen no sooner than after 6 months after treatment implementation, one has to wait for 2-3 years for maximal effect. […] Although clinical response means quality of life improvement, it is hematological remission that influences patients outcome.

• #134 :: JKMS :: Journal of Korean Medical Science

  https://jkms.org/DOIx.php?id=10.3346/jkms.2024.39.e5

  The CRH rate, CRV rate, and clinical improvement rate were 67.2%, 70.0%, and 82.0%, respectively. The median OS was not reached (5-year OS: 78%; 10-year OS: 65%), and the median PFS was 71.4 months (5-year PFS: 55%; 10-year PFS: 37%). […] The diagnosis of POEMS syndrome required keen observation of patients with related symptoms, with a median duration of 173 days from neurological symptoms to diagnosis, which was shorter than that reported previously. […] The treatment outcomes of Korean patients with POEMS syndrome have improved in recent years.

• #135 What is the best first-line treatment for POEMS syndrome: autologous transplantation, melphalan and dexamethasone, or lenalidomide and dexamethasone? | Leukemia

  https://www.nature.com/articles/s41375-019-0391-2

  The total CRH rate was 46.4%. It was higher with ASCT (49.7%) than with MDex (37.7%, p=0.001). The total CRV rate was 55.1%. CRV was significantly better after ASCT than after MDex (38.5%, p=0.001) or LDex (47.7%, p=0.008). The overall 3-year PFS rate was 80.5% and was significantly higher in ASCT (87.6%) than in LDex (64.9%) patients (p=0.003) but was not different from patients in the MDex group (84.6%, p=0.568). […] In conclusion, all three treatments had reasonable responses and survivals in newly diagnosed POEMS syndrome patients. However, ASCT might have better responses and survivals compared with the other two groups, especially for higher risk patients.

• #136 :: JKMS :: Journal of Korean Medical Science

  https://jkms.org/DOIx.php?id=10.3346/jkms.2024.39.e5

  The CRH rate, CRV rate, and clinical improvement rate were 67.2%, 70.0%, and 82.0%, respectively. The median OS was not reached (5-year OS: 78%; 10-year OS: 65%), and the median PFS was 71.4 months (5-year PFS: 55%; 10-year PFS: 37%). […] The diagnosis of POEMS syndrome required keen observation of patients with related symptoms, with a median duration of 173 days from neurological symptoms to diagnosis, which was shorter than that reported previously. […] The treatment outcomes of Korean patients with POEMS syndrome have improved in recent years.

• #137 What is the best first-line treatment for POEMS syndrome: autologous transplantation, melphalan and dexamethasone, or lenalidomide and dexamethasone? | Leukemia

  https://www.nature.com/articles/s41375-019-0391-2

  POEMS syndrome is a rare plasma cell dyscrasia characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes. There is currently no standard treatment. Current systematic therapies include high-dose chemotherapy with autologous stem cell transplantation (ASCT), alkylator-based therapy, and therapy with novel agents. High-dose chemotherapy with ASCT can achieve excellent clinical responses, with a significant improvement of neuropathy, complete hematologic response (CRH) in 60% of POEMS syndrome patients, and complete VEGF response (CRV) in 50%. Five-year progression-free survival (PFS) of 75% and 5-year overall survival (OS) of 90% have been reported. However, treatment-related complications are frequent, with peri-engraftment syndrome reported in 12-50% of patients.

• #138 :: JKMS :: Journal of Korean Medical Science

  https://jkms.org/DOIx.php?id=10.3346/jkms.2024.39.e5

  The CRH rate, CRV rate, and clinical improvement rate were 67.2%, 70.0%, and 82.0%, respectively. The median OS was not reached (5-year OS: 78%; 10-year OS: 65%), and the median PFS was 71.4 months (5-year PFS: 55%; 10-year PFS: 37%). […] The diagnosis of POEMS syndrome required keen observation of patients with related symptoms, with a median duration of 173 days from neurological symptoms to diagnosis, which was shorter than that reported previously. […] The treatment outcomes of Korean patients with POEMS syndrome have improved in recent years.

• #139 :: JKMS :: Journal of Korean Medical Science

  https://jkms.org/DOIx.php?id=10.3346/jkms.2024.39.e5

  The CRH rate, CRV rate, and clinical improvement rate were 67.2%, 70.0%, and 82.0%, respectively. The median OS was not reached (5-year OS: 78%; 10-year OS: 65%), and the median PFS was 71.4 months (5-year PFS: 55%; 10-year PFS: 37%). […] The diagnosis of POEMS syndrome required keen observation of patients with related symptoms, with a median duration of 173 days from neurological symptoms to diagnosis, which was shorter than that reported previously. […] The treatment outcomes of Korean patients with POEMS syndrome have improved in recent years.

• #140 Comprehensive Diagnosis and Management of POEMS Syndrome

  https://pmc.ncbi.nlm.nih.gov/articles/PMC9624442/

  Most patients with POEMS syndrome respond favorably to treatment, but neurological improvement may not commence for 2 years after treatment and may continue for years. Chronic and irreversible functional impairment following treatment is common and requires ongoing monitoring and support. Some is preventable and early provision of occupational and physiotherapy support to maintain passive and active movement, reduce tendon contractures and pain and prevent complications of immobility is invaluable.

• #141

  https://journals.lww.com/hemasphere/fulltext/2022/11000/comprehensive_diagnosis_and_management_of_poems.12.aspx

  Most patients with POEMS syndrome respond favorably to treatment, but neurological improvement may not commence for 2 years after treatment and may continue for years. Chronic and irreversible functional impairment following treatment is common and requires ongoing monitoring and support. Some is preventable and early provision of occupational and physiotherapy support to maintain passive and active movement, reduce tendon contractures and pain and prevent complications of immobility is invaluable.

• #142

  https://journals.lww.com/hemasphere/fulltext/2022/11000/comprehensive_diagnosis_and_management_of_poems.12.aspx

  Most patients with POEMS syndrome respond favorably to treatment, but neurological improvement may not commence for 2 years after treatment and may continue for years. Chronic and irreversible functional impairment following treatment is common and requires ongoing monitoring and support. Some is preventable and early provision of occupational and physiotherapy support to maintain passive and active movement, reduce tendon contractures and pain and prevent complications of immobility is invaluable.

• #143 POEMS syndrome – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/poems-syndrome/symptoms-causes/syc-20352678

  POEMS syndrome is a rare blood disorder that damages nerves and affects other parts of the body. Symptoms may include weak legs, trouble breathing, a larger spleen or liver, and skin changes. […] Without treatment, POEMS syndrome gets worse quickly. It can become life-threatening. So early diagnosis is vital. […] The symptoms of POEMS syndrome make up its name. Symptoms most often appear over weeks to months. They include the following: Polyneuropathy. This may include numbness, tingling and weakness in the legs. In time, it might affect the hands. It also involves trouble breathing. Polyneuropathy must be present for a POEMS diagnosis. Organomegaly. This is an enlarged spleen, liver or lymph nodes. Endocrinopathy and edema. Atypical hormone levels can cause an underactive thyroid, also called hypothyroidism. The hormone levels also can cause diabetes, sexual issues, tiredness, and swelling in arms and legs. Monoclonal protein. This is a protein that atypical plasma cells in bone marrow make. The monoclonal protein goes into the bloodstream. This must be present for the diagnosis of POEMS syndrome. Monoclonal protein often is linked with bones getting harder or thicker. Skin changes. This may involve having more color than usual on the skin. There may be red spots, which may be harder to see on Black or brown skin. The skin might be thicker. There might be more hair on the face or the legs.

• #144 POEMS Syndrome: What It Is, Symptoms, Causes, and Treatment

  https://www.verywellhealth.com/poems-syndrome-7558580

  POEMS syndrome is expected to progress rapidly and can be life-threatening without treatment. Diagnosis and treatment can help your outcome. […] Without treatment, POEMS syndrome is expected to progress and cause more harm throughout the body. Damage to organs that are already affected may worsen, and additional areas of the body can begin to become involved as well. […] Eventually, without treatment, these effects can become life-threatening.

• #145

  https://link.springer.com/article/10.1007/s00415-018-9110-6

  POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, M-protein, skin changes) is a rare paraneoplastic syndrome, caused by a plasma cell proliferative disorder, which is most commonly lambda restricted. The neurological hallmark, which forms one of the mandatory criteria for diagnosis, is a subacute onset demyelinating neuropathy, which can be rapidly disabling and painful. […] Patients typically describe a subacute, painful, distal neuropathy. If POEMS syndrome is suspected, a thorough systemic examination and timely organisation of relevant investigations are required to elicit all features that might aid diagnosis. […] Patients typically present with a subacute, distal, symmetrical, sensorimotor neuropathy, frequently painful, with allodynia and hyperpathia. Neuropathy is a common first clinical feature, and may be the only feature at first presentation.

• #146 Comprehensive Diagnosis and Management of POEMS Syndrome

  https://pmc.ncbi.nlm.nih.gov/articles/PMC9624442/

  Sensorimotor neuropathy is the most common initial symptom of POEMS syndrome. This typically starts distally, in the feet and progresses proximally in a length-dependent fashion; the hands only become affected once neuropathy reaches the knees. Deterioration may occur in days-to-weeks in some and weeks-to-months in others. Patients initially complain of a cramping, tight sensation in the calves initially as if they have run too far and later, bilateral, hyperesthetic and dysaesthetic neuropathic pain. Weakness is striking in its distal selectivity (below the ankles and the intrinsic hand muscles) and differs from chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) in its lack of proximal weakness for the earlier course. Early increased tendency to trip over feet may be due to footdrop.

• #147 Recent Advances in the Treatment and Supportive Care of POEMS Syndrome

  https://www.mdpi.com/2077-0383/11/23/7011

  POEMS is a rare clonal plasma cell disorder characterized by multi-systemic features that include demyelinating peripheral neuropathy, organomegaly, endocrinopathy, presence of monoclonal proteins (M-protein), and skin changes. […] When diagnosed and treated early, the median survival of patients is 13.7 years. Yet, if the disease is left untreated, it can progress to affect the respiratory and nervous systems at a higher degree and can be fatal. […] The diagnosis of POEMS is mostly clinical, complemented by laboratory studies and radiographic imaging. Any sign or symptom should elicit an in-depth investigation for POEMS particularly in a patient with monoclonal gammopathy and peripheral neuropathy. […] The differential diagnoses to keep in mind are chronic inflammatory polyradiculoneuropathy (CIPD), anti-myelin-associated-glycoprotein (MAG) neuropathy, monoclonal gammopathy of undetermined significance (MGUS), and immunoglobulin light chain (AL) amyloid neuropathy.

• #148

  https://journals.lww.com/hemasphere/fulltext/2022/11000/comprehensive_diagnosis_and_management_of_poems.12.aspx

  These clinical features alongside a monoclonal plasma cell disorderalmost always lambda light-chain restrictedor unexplained polycythemia or thrombocytosis should prompt investigation for other POEMS features. Peripheral edema is found in most and may present as ascites (7%54%), pleural (3%43%), and pericardial effusions (1%64%). This is often poorly responsive to diuretics as the excess fluid is not within the vascular compartment. Papilledema, sometimes with characteristic subretinal hemorrhages, and without raised intracranial pressure is present in 30%40% of patients. Stroke is a recognized feature, and noninfiltrative, noninfective pachymeningitis caused by meningeal fluid leak has been recently reported in 70% of cases. […] Skin lesions are common (70%). Glomerular hemangiomata are a pathognomonic finding, but sclerodermoid changes of the digits (5%43%), temperature-sensitive blue/purple discoloration of the feet (acrocyanosis 19%), leukonychia, clubbing (5%49%), hyperpigmentation of the skin (46%93%), and new growth of excess bodily hair (26%74%) are frequent and readily visible. Other features suggestive of POEMS include fatigue, unexplained weight loss, enlarged lymph nodes or organomegaly, unprovoked venous and/or arterial thrombosis and features of gonadal failure (erectile dysfunction, early menopausal symptoms, infertility).

• #149 Recent Advances in the Treatment and Supportive Care of POEMS Syndrome

  https://www.mdpi.com/2077-0383/11/23/7011

  POEMS is a rare clonal plasma cell disorder characterized by multi-systemic features that include demyelinating peripheral neuropathy, organomegaly, endocrinopathy, presence of monoclonal proteins (M-protein), and skin changes. […] When diagnosed and treated early, the median survival of patients is 13.7 years. Yet, if the disease is left untreated, it can progress to affect the respiratory and nervous systems at a higher degree and can be fatal. […] The diagnosis of POEMS is mostly clinical, complemented by laboratory studies and radiographic imaging. Any sign or symptom should elicit an in-depth investigation for POEMS particularly in a patient with monoclonal gammopathy and peripheral neuropathy. […] The differential diagnoses to keep in mind are chronic inflammatory polyradiculoneuropathy (CIPD), anti-myelin-associated-glycoprotein (MAG) neuropathy, monoclonal gammopathy of undetermined significance (MGUS), and immunoglobulin light chain (AL) amyloid neuropathy.

• #150 Recent Advances in the Treatment and Supportive Care of POEMS Syndrome

  https://www.mdpi.com/2077-0383/11/23/7011

  Due to the debilitating nature of POEMS syndrome, early detection and intervention is critical to mitigate the risk of permanent neurologic disability. […] The main goal of treatment is to inactivate the plasma cell responsible for this syndrome with the additional goals of preserving and potentially reversing organ dysfunction. […] POEMS syndrome is a disease chronic in nature, in which the polyneuropathy can be progressive in nature and correlates with the activity of the plasma cell clone. […] A retrospective study revealed that the median survival of the patients with POEMS was 165 months, regardless of how many features of POEMS syndrome are present at the time of diagnosis. However, the patients who, after treatment, still had nail clubbing had a median survival of only 31 months and the patients with extravascular fluid overload had a median survival of 79 months with the two variables being independent of each other.

• #151 Treatment and outcomes of POEMS syndrome: changes in the past 20 years | Blood Cancer Journal

  https://www.nature.com/articles/s41408-021-00540-1

  Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome is a rare plasma cell dyscrasia with multiorgan involvement. […] The time interval from symptom onset to diagnosis was 23.0 in the entire cohort and 15.9, 12.6, and 12.2 months in the three cohorts. […] Neuropathy was less severe in 2015-2019 than in the other periods, and 35.8, 41.7, and 29.6% of the patients in each group were evaluated as having Overall Neuropathy Limitations Scale (ONLS)4. […] The incidence of hepatomegaly and splenomegaly significantly decreased over time, as well as symptoms related to extravascular volume overload, such as peripheral edema and pleural effusion. […] The incidence of skin changes, such as angioma and hypertrichosis, increased, which may be attributed to the increased understanding of the disease and more careful physical evaluation. […] The 5-year OS rates in the three periods were 70.0, 85.4, and 92.2%. […] Overall survival increased significantly, mainly due to dramatic advances in non-ASCT therapeutic options.

• #152 Get POEMS Syndrome Treatment | Cleveland Clinic

  https://my.clevelandclinic.org/services/poems-syndrome-treatment

  It seems like the tingling in your arms and legs is getting worse. […] This numbness, along with other symptoms, could be signs of a rare (but treatable) blood condition called POEMS syndrome. […] POEMS syndrome, a chronic (ongoing) blood condition, starts when your body makes abnormal plasma cells. These cells start to multiply out of control, damaging your organs and nerves. POEMS syndrome stands for the signs you may have: Polyneuropathy: Nerve damage throughout your body causes pain, tingling and numbness. […] Diagnosing POEMS syndrome isnt always simple. While everyone with POEMS has polyneuropathy and an M-protein, other symptoms vary and can be similar to different neurological conditions. […] Its important to understand that POEMS syndrome affects everyone differently. The ways you can get to remission (no flare-ups) can be quite different, too. […] Cleveland Clinics healthcare providers will help you manage your POEMS syndrome symptoms with advanced treatments and ongoing support.

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