Materiały źródłowe

• #1 How I treat POEMS syndrome

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3425020/

  POEMS syndrome is a paraneoplastic syndrome whose acronym stands for less than half of the defining features of the disease, that is, polyradiculoneuropathy, organomegaly, potentially including coexisting Castleman disease, endocrinopathy, monoclonal plasma cell neoplasm, and skin changes. […] The diagnosis is based on having both the polyradiculoneuropathy and the monoclonal plasma cell disorder, and at least 1 of the other 3 major criteria (Castleman disease, sclerotic bone lesions, or elevated VEGF) and at least one minor criterion. […] Prompt treatment directed at the underlying plasma cell clone produces dramatic responses in the majority of patients. […] Although there are no randomized clinical trial data to direct best therapy, for patients with disseminated disease, high-dose chemotherapy with peripheral blood transplantation has yielded durable benefit, whereas radiation therapy is typically effective for patients with a more localized presentation.

• #2 Recent Advances in the Treatment and Supportive Care of POEMS Syndrome – PubMed

  https://pubmed.ncbi.nlm.nih.gov/36498588/

  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. […] The key is early diagnosis and immediate anti-plasma cell directed therapy for the best clinical outcomes. […] For patients with disseminated disease as defined by bone marrow involvement or more than three osteosclerotic bone lesions, high-dose chemotherapy with autologous hematopoietic stem cell transplant (ASCT) yields durable responses and is the preferred treatment in eligible patients. […] For patients with localized bony disease, radiotherapy has proven to be very effective. […] Lenalidomide and dexamethasone is a proven therapy in patients ineligible for ASCT. […] In this review article, we tackle the diagnostic approach and discuss the latest treatment modalities of this rare debilitating disease.

• #3 POEMS Syndrome | Condition | UAMS Health

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

  POEMS syndrome is a rare, multi-system blood disorder. […] Treatment is aimed at the underlying plasma cell disorder and relieving symptoms. Every patients disease is different. Myeloma Center physicians are dedicated to defining the nuances of each individuals disease and customizing treatment regimens that have the most promise for effective results. […] Patients with POEMS syndrome often need treatment to alleviate the symptoms of neuropathy, extravascular volume overload, and endocrine abnormalities. […] A multi-disciplinary approach to the treatment of POEMS syndrome is essential. The physician team should include myeloma specialists, radiologists, neurologists, nephrologists, cardiologists and other specialists as needed.

• #4 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. […] The key is early diagnosis and immediate anti-plasma cell directed therapy for the best clinical outcomes. […] For patients with disseminated disease as defined by bone marrow involvement or more than three osteosclerotic bone lesions, high-dose chemotherapy with autologous hematopoietic stem cell transplant (ASCT) yields durable responses and is the preferred treatment in eligible patients. […] The treatment of POEMS syndrome can be divided into two major categories: targeting the clone by specific anti-plasma cell directed therapy and targeting the symptoms with symptomatic and supportive care.

• #5

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

  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. […] Supportive care considerations include management of endocrinopathy, neuropathy, thrombosis, and infection. […] Effective management requires targeting the underlying plasma cell clone in parallel with proactive management of comorbid risks and POEMS-induced physiological impairments. […] The aim of treatment is to eradicate the underlying plasma cell dyscrasia, but there are limited trial data to guide treatment decisions. […] Clinical nurse specialists or equivalent should act as a keyworker and signpost patients and family members to sources of information and support.

• #6 POEMS Syndrome | Condition | UAMS Health

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

  POEMS syndrome is a rare, multi-system blood disorder. […] Treatment is aimed at the underlying plasma cell disorder and relieving symptoms. Every patients disease is different. Myeloma Center physicians are dedicated to defining the nuances of each individuals disease and customizing treatment regimens that have the most promise for effective results. […] Patients with POEMS syndrome often need treatment to alleviate the symptoms of neuropathy, extravascular volume overload, and endocrine abnormalities. […] A multi-disciplinary approach to the treatment of POEMS syndrome is essential. The physician team should include myeloma specialists, radiologists, neurologists, nephrologists, cardiologists and other specialists as needed.

• #7 POEMS syndrome | University of Iowa Health Care

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

  POEMS syndrome patients experience numbness, tingling, and weakness in the toes, feet, and later the hands. […] Doctors take a two-pronged approach to treatment. First, they target the M in POEMSthe growth of abnormal plasma cells in the bloodas the underlying cause of the disease. Second, they work to relieve the symptoms caused by the disease. […] Each patients case is different, and doctors will choose a course of treatment that shows the greatest promise for effective results for their individual patient. […] Other treatments to target the abnormal plasma cells may include: Physical therapy to strengthen legs and combat the effects of neuropathy. […] POEMS syndrome patients can be effectively treated and return to an overall normal quality of life. The key for addressing the many facets of POEMS syndrome is being cared for by a team of specialists, including myeloma specialists, radiologists, neurologists, nephrologists, cardiologists, physical therapists, pharmacists, and others.

• #8

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

  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. […] Supportive care considerations include management of endocrinopathy, neuropathy, thrombosis, and infection. […] Effective management requires targeting the underlying plasma cell clone in parallel with proactive management of comorbid risks and POEMS-induced physiological impairments. […] The aim of treatment is to eradicate the underlying plasma cell dyscrasia, but there are limited trial data to guide treatment decisions. […] Clinical nurse specialists or equivalent should act as a keyworker and signpost patients and family members to sources of information and support.

• #9 How I treat POEMS syndrome

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3425020/

  Papilledema (optic disc edema) is present in at least one-third of patients and may be associated with increased intracranial pressure. […] The neuropathy is usually the dominant characteristic. […] Patients with POEMS syndrome present with a complex conglomerate of symptoms, signs, and objective abnormalities, making the diagnosis, management, and follow-up a challenge. […] Early diagnosis and a prompt multidisciplinary approach increase the likelihood of reduced long-term irreversible morbidity. […] Both therapies directed at other features of the disease as well as emotional support should be a major part of the care plan. […] Serial assessments of clinical stigmata (peripheral neuropathy, volume status, eyes, skin, and organomegaly) of blood (M-spike, VEGF, affected endocrine parameters) should be done every 3 months for at least the first several years.

• #10 Get POEMS Syndrome Treatment | Cleveland Clinic

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

  POEMS syndrome is so rare that few doctors know about it. But its not rare at Cleveland Clinic. Were at the forefront of innovative POEMS syndrome diagnosis and care. Our healthcare providers know what it takes to detect signs that are easy to miss. Theyll confirm a diagnosis and get you started with treatment right away. […] Cleveland Clinic offers the POEMS syndrome tests, treatments and support you need to get relief. Your care team of providers from different specialties focus on you and tailor treatment to your unique diagnosis and needs. […] Our experience with POEMS syndrome has helped many people with severe symptoms feel better. Theyre back on their feet, working and spending quality time with loved ones. […] Cleveland Clinic is a world leader in autologous stem cell transplants, a common POEMS syndrome treatment. This treatment uses a sample of your blood-forming (stem) cells to develop healthy plasma, which will give you relief from ongoing POEMS symptoms.

• #11 Get POEMS Syndrome Treatment | Cleveland Clinic

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

  Cleveland Clinic believes a team-based approach to care gives you the most personalized treatment and support. Your care team will include healthcare providers from different specialties based on your unique diagnosis and needs. […] 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. […] We use what we learn from your health history, physical exam and test results to make sure you get the most personalized care. You may have only one type of treatment to manage your condition, or you may need a combination of different ones, like: […] Our expert physical therapists focus exercises on nerve weakness to help you get stronger and more mobile. […] Your care team may recommend you have radiation to destroy abnormal plasma cells if theyre found in limited spots.

• #12 How I treat POEMS syndrome

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3425020/

  Treatment of the POEMS syndrome can be broken down into 2 major categories: targeting the underlying clone and targeting the rest of the syndrome. […] The requirements set forth in Table 1 are designed to retain both sensitivity and specificity, potentially erring on the side of specificity. […] The 2 best ways to approach the peripheral neuropathy are to target the clone and to direct the patient to work intensively with physical therapy and occupational therapy. […] I treat the painful peripheral neuropathy if present with drugs, such as gabapentin, pregabalin, amitriptyline, nortriptyline, duloxetine, topical lidocaine patches, and topical ketamine, lidocaine, and amitriptyline compounds. […] The hepatosplenomegaly and lymphadenopathy do not require specific therapy. […] Endocrinopathy is a central but poorly understood feature of POEMS.

• #13 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. […] The key is early diagnosis and immediate anti-plasma cell directed therapy for the best clinical outcomes. […] For patients with disseminated disease as defined by bone marrow involvement or more than three osteosclerotic bone lesions, high-dose chemotherapy with autologous hematopoietic stem cell transplant (ASCT) yields durable responses and is the preferred treatment in eligible patients. […] The treatment of POEMS syndrome can be divided into two major categories: targeting the clone by specific anti-plasma cell directed therapy and targeting the symptoms with symptomatic and supportive care.

• #14

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

  Evidence for treatment in POEMS syndrome is largely limited to retrospective cohort studies, with only one randomised controlled trial (RCT) to date. IVIg or steroid monotherapy, commonly used in other inflammatory neuropathies, does not produce lasting benefit. […] The current suggested treatment algorithm recommends localised radiotherapy for patients with localised disease, defined as up to 3 discrete bone lesions and no evidence of clonal plasma cells on iliac crest biopsy, or systemic treatment in patients with diffuse disease, defined as 3 bone lesions or clonal plasma cells on iliac crest biopsy. […] Supportive treatment for neurological disability and systemic symptoms should also be considered. […] Neurological symptoms have been shown to improve post-ASCT. In a study of 60 patients followed up over a median 61 months, wheelchair use improved from 45 to 0% post-transplant and there was a reduction in the number of patients requiring walking aids and ankle-foot orthoses.

• #15 Recent Advances in the Treatment and Supportive Care of POEMS Syndrome – PubMed

  https://pubmed.ncbi.nlm.nih.gov/36498588/

  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. […] The key is early diagnosis and immediate anti-plasma cell directed therapy for the best clinical outcomes. […] For patients with disseminated disease as defined by bone marrow involvement or more than three osteosclerotic bone lesions, high-dose chemotherapy with autologous hematopoietic stem cell transplant (ASCT) yields durable responses and is the preferred treatment in eligible patients. […] For patients with localized bony disease, radiotherapy has proven to be very effective. […] Lenalidomide and dexamethasone is a proven therapy in patients ineligible for ASCT. […] In this review article, we tackle the diagnostic approach and discuss the latest treatment modalities of this rare debilitating disease.

• #16

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

  Evidence for treatment in POEMS syndrome is largely limited to retrospective cohort studies, with only one randomised controlled trial (RCT) to date. IVIg or steroid monotherapy, commonly used in other inflammatory neuropathies, does not produce lasting benefit. […] The current suggested treatment algorithm recommends localised radiotherapy for patients with localised disease, defined as up to 3 discrete bone lesions and no evidence of clonal plasma cells on iliac crest biopsy, or systemic treatment in patients with diffuse disease, defined as 3 bone lesions or clonal plasma cells on iliac crest biopsy. […] Supportive treatment for neurological disability and systemic symptoms should also be considered. […] Neurological symptoms have been shown to improve post-ASCT. In a study of 60 patients followed up over a median 61 months, wheelchair use improved from 45 to 0% post-transplant and there was a reduction in the number of patients requiring walking aids and ankle-foot orthoses.

• #17 How I treat POEMS syndrome

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3425020/

  POEMS syndrome is a paraneoplastic syndrome whose acronym stands for less than half of the defining features of the disease, that is, polyradiculoneuropathy, organomegaly, potentially including coexisting Castleman disease, endocrinopathy, monoclonal plasma cell neoplasm, and skin changes. […] The diagnosis is based on having both the polyradiculoneuropathy and the monoclonal plasma cell disorder, and at least 1 of the other 3 major criteria (Castleman disease, sclerotic bone lesions, or elevated VEGF) and at least one minor criterion. […] Prompt treatment directed at the underlying plasma cell clone produces dramatic responses in the majority of patients. […] Although there are no randomized clinical trial data to direct best therapy, for patients with disseminated disease, high-dose chemotherapy with peripheral blood transplantation has yielded durable benefit, whereas radiation therapy is typically effective for patients with a more localized presentation.

• #18 POEMS Syndrome Treatment & Management: Medical Care, Surgical Care, Consultations

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

  Treatment with high-dose chemotherapy followed by autologous peripheral blood stem cell transplantation may result in clinical improvement of polyneuropathy. […] Relapse after successful autologous peripheral blood stem cell transplantation has been reported. […] A 2014 multicenter retrospective study in Japan showed positive results for autologous stem cell transplantation in the treatment of patients with POEMS syndrome in terms of long-term survival and quality of life. […] Even if the plasmacytoma shows a complete response to treatment, patients still need long-term follow-up care because some symptoms (eg, neurologic defects and functional loss due to tightening of the skin) may be permanent.

• #19 Recent Advances in the Treatment and Supportive Care of POEMS Syndrome – PubMed

  https://pubmed.ncbi.nlm.nih.gov/36498588/

  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. […] The key is early diagnosis and immediate anti-plasma cell directed therapy for the best clinical outcomes. […] For patients with disseminated disease as defined by bone marrow involvement or more than three osteosclerotic bone lesions, high-dose chemotherapy with autologous hematopoietic stem cell transplant (ASCT) yields durable responses and is the preferred treatment in eligible patients. […] For patients with localized bony disease, radiotherapy has proven to be very effective. […] Lenalidomide and dexamethasone is a proven therapy in patients ineligible for ASCT. […] In this review article, we tackle the diagnostic approach and discuss the latest treatment modalities of this rare debilitating disease.

• #20 POEMS Syndrome: Still an Enigma?

  https://www.cancernetwork.com/view/poems-syndrome-still-enigma

  The POEMS syndrome treatment guidelines proposed by Drs. Dispenzieri and Buadi, based on the number of osteosclerotic bone lesions and the presence or absence of bone marrow infiltration, are very easy to use. Examination of the bone marrow is critical to determine whether infiltration of bone has occurred, given that irradiation is essential if a bone lesion is found. In the case of localized disease, irradiation may induce resolution of symptoms and, eventually, cure. In contrast, when irradiation is not possible, high-dose chemotherapy with stem cell support is the treatment of choice in fit patients, and achieves a nearly 100% response. Alternatively, drugs developed for the treatment of plasmacytic proliferation may be used. Lenalidomide seems to be the more promising therapy, because of its anti-proliferative and anti-angiogenic effects and lack of major neurotoxicity. In small series, a significant proportion of patients have responded to this treatment.

• #21 Bortezomib, ASCT May Improve Survival Outcomes for Patients With POEMS Syndrome – Hematology Advisor

  https://www.hematologyadvisor.com/news/poems-syndrome-bortezomib-asct-improve-survival-outcomes/

  Researchers sought to determine whether treatment with bortezomib and autologous stem cell transplant may improve outcomes in patients with POEMS syndrome. […] Among patients with polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes (POEMS) syndrome, bortezomib and autologous stem cell transplant (ASCT) may improve survival outcomes, according to research published in Clinical Lymphoma, Myeloma Leukemia. […] Given a paucity of research into POEMS syndrome, furthermore, it is difficult to target treatment at the individual level. […] Overall, 37% of patients received a proteasome inhibitor. Patients who received bortezomib monotherapy had the highest rates of complete or very good partial remission (69%). […] Among the 30% of patients who had a planned ASCT as part of frontline treatment, both progression-free survival and overall survival (OS) were improved (P =.003). […] Proteasome inhibitors as single agents, the combination of a [proteasome inhibitor and immunomodulatory agent] and ASCT all demonstrate high responses and should be considered standard options for a newly diagnosed POEMS patient.

• #22 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. […] The key is early diagnosis and immediate anti-plasma cell directed therapy for the best clinical outcomes. […] For patients with disseminated disease as defined by bone marrow involvement or more than three osteosclerotic bone lesions, high-dose chemotherapy with autologous hematopoietic stem cell transplant (ASCT) yields durable responses and is the preferred treatment in eligible patients. […] The treatment of POEMS syndrome can be divided into two major categories: targeting the clone by specific anti-plasma cell directed therapy and targeting the symptoms with symptomatic and supportive care.

• #23 How I treat POEMS syndrome

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3425020/

  Treatment of the POEMS syndrome can be broken down into 2 major categories: targeting the underlying clone and targeting the rest of the syndrome. […] The requirements set forth in Table 1 are designed to retain both sensitivity and specificity, potentially erring on the side of specificity. […] The 2 best ways to approach the peripheral neuropathy are to target the clone and to direct the patient to work intensively with physical therapy and occupational therapy. […] I treat the painful peripheral neuropathy if present with drugs, such as gabapentin, pregabalin, amitriptyline, nortriptyline, duloxetine, topical lidocaine patches, and topical ketamine, lidocaine, and amitriptyline compounds. […] The hepatosplenomegaly and lymphadenopathy do not require specific therapy. […] Endocrinopathy is a central but poorly understood feature of POEMS.

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

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

  Endocrinopathy is central in the disease process with hypogonadism being the most common abnormality followed by different endocrinopathies such as thyroid dysfunction, glucose metabolism abnormalities, adrenal insufficiency, and hyperprolactinemia. […] The morbidity and mortality can be reduced with an early diagnosis and prompt treatment. […] The follow up of patients should be completed at least every 3 months for at least several years.

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

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

  Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin abnormalities (POEMS) syndrome is a rare multisystem disease that causes a blood cell disorder and nerve damage, with several other effects. […] There is no cure for POEMS syndrome, but treatments can help alleviate some of the effects. […] Individualized treatments can alleviate specific effects, such as diabetes, thyroid disease, or pain from polyneuropathy. […] Your healthcare team may also prescribe steroids or diuretics (water pills) to ease swelling. […] Without treatment, POEMS syndrome is expected to progress and cause more harm throughout the body. […] Diagnosis and treatment can help your outcome. […] If you or a loved one is living with POEMS syndrome, you must seek medical care from a healthcare team with experience in this condition and learn about maintaining the best possible quality of life.

• #26 POEMS Syndrome – Treatment abroad

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

  Since the cause of POEMS syndrome is currently unknown, there is no standardized treatment for causation; however, the need for pretreatment of the plasma cell proliferative disorder is now being elucidated. […] Therapy with high doses of intravenous immunoglobulin and plasmapheresis does not always give the desired effect.

• #27 How I treat POEMS syndrome

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3425020/

  Treatment of the POEMS syndrome can be broken down into 2 major categories: targeting the underlying clone and targeting the rest of the syndrome. […] The requirements set forth in Table 1 are designed to retain both sensitivity and specificity, potentially erring on the side of specificity. […] The 2 best ways to approach the peripheral neuropathy are to target the clone and to direct the patient to work intensively with physical therapy and occupational therapy. […] I treat the painful peripheral neuropathy if present with drugs, such as gabapentin, pregabalin, amitriptyline, nortriptyline, duloxetine, topical lidocaine patches, and topical ketamine, lidocaine, and amitriptyline compounds. […] The hepatosplenomegaly and lymphadenopathy do not require specific therapy. […] Endocrinopathy is a central but poorly understood feature of POEMS.

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

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

  During treatment, hematologic response is monitored by measuring the M-protein concentration and the serum VEGF levels. […] The best way to target peripheral neuropathy is by targeting the clone by anti-plasma cell therapy. […] Neurorehabilitation is an essential part of the treatment plan, and it is essential for sensory as well as motor impairment of the patient. […] The mainstay of treatment is anti-plasma cell therapy such as IMiD or ASCT which will relieve polyneuropathy in most if not all of patients over times. […] The majority of patients will present with signs of fluid overload and anasarca, mainly attributed to the activity of serum VEGF and its contribution to capillary leak. […] Therapy to renal disease specifically is not established, but targeting the clone is the mainstay of treatment with an overall alleviation of signs and symptoms.

• #29 How I treat POEMS syndrome

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3425020/

  Treatment of the POEMS syndrome can be broken down into 2 major categories: targeting the underlying clone and targeting the rest of the syndrome. […] The requirements set forth in Table 1 are designed to retain both sensitivity and specificity, potentially erring on the side of specificity. […] The 2 best ways to approach the peripheral neuropathy are to target the clone and to direct the patient to work intensively with physical therapy and occupational therapy. […] I treat the painful peripheral neuropathy if present with drugs, such as gabapentin, pregabalin, amitriptyline, nortriptyline, duloxetine, topical lidocaine patches, and topical ketamine, lidocaine, and amitriptyline compounds. […] The hepatosplenomegaly and lymphadenopathy do not require specific therapy. […] Endocrinopathy is a central but poorly understood feature of POEMS.

• #30 Get POEMS Syndrome Treatment | Cleveland Clinic

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

  Cleveland Clinic believes a team-based approach to care gives you the most personalized treatment and support. Your care team will include healthcare providers from different specialties based on your unique diagnosis and needs. […] 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. […] We use what we learn from your health history, physical exam and test results to make sure you get the most personalized care. You may have only one type of treatment to manage your condition, or you may need a combination of different ones, like: […] Our expert physical therapists focus exercises on nerve weakness to help you get stronger and more mobile. […] Your care team may recommend you have radiation to destroy abnormal plasma cells if theyre found in limited spots.

• #31

  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. […] Provision of in-bed or in-chair exercises, mobility aids, and adaptations have to adjust to the changing needs of the patient. […] The goal of treatment in POEMS syndrome is to suppress the underlying plasma cell clone, but although in some cases it is possible, complete disease suppression (CRH) is not always achievable particularly in frail patients. […] We perform disease evaluation at six months or sooner if there are concerns about an inadequate response. […] Patients with known endocrinopathy should undergo regular clinical and biochemical review of endocrine function, particularly as dysfunction can have major effects on bone health and quality of life.

• #32 POEMS syndrome – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/poems-syndrome/diagnosis-treatment/drc-20582628

  Our caring team of Mayo Clinic experts can help you with your poems syndrome-related health concerns […] Treatment for POEMS syndrome might ease symptoms but doesn’t cure the condition. Treatments include radiation therapy, chemotherapy and stem cell transplant. […] Physical therapy. This can help with the weakness and other symptoms of polyneuropathy. […] POEMS syndrome care at Mayo Clinic

• #33 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/

  POEMS syndrome patient Chukwuma Anyanwu and his physician, Claudia Paba-Prada, MD, and his nurse, Kathleen Finn, RN. […] Anyanwus recovery has been so dramatic that he now works out at a gym every day, drives his car, and hopes to return to his job at a bank in a few weeks. […] He always thought he would get better, even when I couldnt promise that he would. -Anyanwus physician, Claudia Paba-Prada, MD. […] A visiting nurse checked him every day, but nothing relieved the burning, shooting pains. […] He went on medical leave from his job as an operations analyst at a bank; his family moved from a third-floor residence to a ground-floor one, which had been modified for accessibility; he needed help eating and bathing; he moved about his home in a motorized wheelchair. […] Following radiation treatment, he began receiving the chemotherapy drug Revlimid in combination with steroids.

• #34

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

  Evidence for treatment in POEMS syndrome is largely limited to retrospective cohort studies, with only one randomised controlled trial (RCT) to date. IVIg or steroid monotherapy, commonly used in other inflammatory neuropathies, does not produce lasting benefit. […] The current suggested treatment algorithm recommends localised radiotherapy for patients with localised disease, defined as up to 3 discrete bone lesions and no evidence of clonal plasma cells on iliac crest biopsy, or systemic treatment in patients with diffuse disease, defined as 3 bone lesions or clonal plasma cells on iliac crest biopsy. […] Supportive treatment for neurological disability and systemic symptoms should also be considered. […] Neurological symptoms have been shown to improve post-ASCT. In a study of 60 patients followed up over a median 61 months, wheelchair use improved from 45 to 0% post-transplant and there was a reduction in the number of patients requiring walking aids and ankle-foot orthoses.

• #35

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

  Evidence for treatment in POEMS syndrome is largely limited to retrospective cohort studies, with only one randomised controlled trial (RCT) to date. IVIg or steroid monotherapy, commonly used in other inflammatory neuropathies, does not produce lasting benefit. […] The current suggested treatment algorithm recommends localised radiotherapy for patients with localised disease, defined as up to 3 discrete bone lesions and no evidence of clonal plasma cells on iliac crest biopsy, or systemic treatment in patients with diffuse disease, defined as 3 bone lesions or clonal plasma cells on iliac crest biopsy. […] Supportive treatment for neurological disability and systemic symptoms should also be considered. […] Neurological symptoms have been shown to improve post-ASCT. In a study of 60 patients followed up over a median 61 months, wheelchair use improved from 45 to 0% post-transplant and there was a reduction in the number of patients requiring walking aids and ankle-foot orthoses.

• #36 POEMS | The Foundation for Peripheral Neuropathy

  https://www.foundationforpn.org/causes/poems/

  POEMS syndrome is a rare multisystem disorder caused by the improper growth of bone marrow cells, resulting in an abnormal accumulation of proteins (immunoglobulin) in tissues and organs. […] Treatment focuses on alleviating the worst symptom of POEMS, which is the nerve damage that causes progressive weakness in the body’s extremities. Treatment also attempts to stop the production of the bone marrow cells that create problems in other parts of the body. […] For peripheral neuropathy induced by POEMS: Discontinue drugs that cause peripheral neuropathy. Pain medication. Institute non-drug treatments to reduce pain, such as: Avoiding extended periods of standing or walking. Wearing looser shoes. Take safety measures to compensate for loss of sensation. Ask your doctor about special therapeutic shoes (which may be covered by Medicare and other insurance).

• #37 POEMS | The Foundation for Peripheral Neuropathy

  https://www.foundationforpn.org/causes/poems/

  POEMS syndrome is a rare multisystem disorder caused by the improper growth of bone marrow cells, resulting in an abnormal accumulation of proteins (immunoglobulin) in tissues and organs. […] Treatment focuses on alleviating the worst symptom of POEMS, which is the nerve damage that causes progressive weakness in the body’s extremities. Treatment also attempts to stop the production of the bone marrow cells that create problems in other parts of the body. […] For peripheral neuropathy induced by POEMS: Discontinue drugs that cause peripheral neuropathy. Pain medication. Institute non-drug treatments to reduce pain, such as: Avoiding extended periods of standing or walking. Wearing looser shoes. Take safety measures to compensate for loss of sensation. Ask your doctor about special therapeutic shoes (which may be covered by Medicare and other insurance).

• #38 How I treat POEMS syndrome

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3425020/

  Papilledema (optic disc edema) is present in at least one-third of patients and may be associated with increased intracranial pressure. […] The neuropathy is usually the dominant characteristic. […] Patients with POEMS syndrome present with a complex conglomerate of symptoms, signs, and objective abnormalities, making the diagnosis, management, and follow-up a challenge. […] Early diagnosis and a prompt multidisciplinary approach increase the likelihood of reduced long-term irreversible morbidity. […] Both therapies directed at other features of the disease as well as emotional support should be a major part of the care plan. […] Serial assessments of clinical stigmata (peripheral neuropathy, volume status, eyes, skin, and organomegaly) of blood (M-spike, VEGF, affected endocrine parameters) should be done every 3 months for at least the first several years.

• #39 How I treat POEMS syndrome

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3425020/

  Papilledema (optic disc edema) is present in at least one-third of patients and may be associated with increased intracranial pressure. […] The neuropathy is usually the dominant characteristic. […] Patients with POEMS syndrome present with a complex conglomerate of symptoms, signs, and objective abnormalities, making the diagnosis, management, and follow-up a challenge. […] Early diagnosis and a prompt multidisciplinary approach increase the likelihood of reduced long-term irreversible morbidity. […] Both therapies directed at other features of the disease as well as emotional support should be a major part of the care plan. […] Serial assessments of clinical stigmata (peripheral neuropathy, volume status, eyes, skin, and organomegaly) of blood (M-spike, VEGF, affected endocrine parameters) should be done every 3 months for at least the first several years.

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

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

  Endocrinopathy is central in the disease process with hypogonadism being the most common abnormality followed by different endocrinopathies such as thyroid dysfunction, glucose metabolism abnormalities, adrenal insufficiency, and hyperprolactinemia. […] The morbidity and mortality can be reduced with an early diagnosis and prompt treatment. […] The follow up of patients should be completed at least every 3 months for at least several years.

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

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

  Endocrinopathy is central in the disease process with hypogonadism being the most common abnormality followed by different endocrinopathies such as thyroid dysfunction, glucose metabolism abnormalities, adrenal insufficiency, and hyperprolactinemia. […] The morbidity and mortality can be reduced with an early diagnosis and prompt treatment. […] The follow up of patients should be completed at least every 3 months for at least several years.

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

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

  During treatment, hematologic response is monitored by measuring the M-protein concentration and the serum VEGF levels. […] The best way to target peripheral neuropathy is by targeting the clone by anti-plasma cell therapy. […] Neurorehabilitation is an essential part of the treatment plan, and it is essential for sensory as well as motor impairment of the patient. […] The mainstay of treatment is anti-plasma cell therapy such as IMiD or ASCT which will relieve polyneuropathy in most if not all of patients over times. […] The majority of patients will present with signs of fluid overload and anasarca, mainly attributed to the activity of serum VEGF and its contribution to capillary leak. […] Therapy to renal disease specifically is not established, but targeting the clone is the mainstay of treatment with an overall alleviation of signs and symptoms.

• #43

  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. […] Provision of in-bed or in-chair exercises, mobility aids, and adaptations have to adjust to the changing needs of the patient. […] The goal of treatment in POEMS syndrome is to suppress the underlying plasma cell clone, but although in some cases it is possible, complete disease suppression (CRH) is not always achievable particularly in frail patients. […] We perform disease evaluation at six months or sooner if there are concerns about an inadequate response. […] Patients with known endocrinopathy should undergo regular clinical and biochemical review of endocrine function, particularly as dysfunction can have major effects on bone health and quality of life.

• #44 Pulmonary manifestations of POEM syndrome: a retrospective analysis of 282 cases | BMC Pulmonary Medicine | Full Text

  https://bmcpulmmed.biomedcentral.com/articles/10.1186/s12890-023-02741-9

  Abnormalities in pulmonary manifestations constitute the significant features of POEMS syndrome. Several patients with POEMS syndrome presented with respiratory symptoms at the initial evaluation. These findings underscore the importance of early identification and accurate diagnosis of POEMS syndrome by clinicians, particularly in cases involving lung and multisystem. […] Respiratory symptoms were found in 40.1% of the patients, with dyspnea as the most common symptom (34.4%). […] Patients with respiratory symptoms exhibited higher declined lung function measures than those having no respiratory symptoms. High-risk patients with poor prognosis showed more pulmonary function abnormalities. […] Patients with respiratory symptoms had lower values of lung function, including FEV1, FEV1%, FVC, FVC%, DLCO%, and TLC% than those without respiratory symptoms. […] Consequently, presence of respiratory symptom warrants PFTs and echocardiography. Clinicians should intensify their awareness of the association between POEMS syndrome and pulmonary disease to enhance their understanding of the syndrome for improved diagnosis and treatment.

• #45 Pulmonary manifestations of POEM syndrome: a retrospective analysis of 282 cases | BMC Pulmonary Medicine | Full Text

  https://bmcpulmmed.biomedcentral.com/articles/10.1186/s12890-023-02741-9

  Abnormalities in pulmonary manifestations constitute the significant features of POEMS syndrome. Several patients with POEMS syndrome presented with respiratory symptoms at the initial evaluation. These findings underscore the importance of early identification and accurate diagnosis of POEMS syndrome by clinicians, particularly in cases involving lung and multisystem. […] Respiratory symptoms were found in 40.1% of the patients, with dyspnea as the most common symptom (34.4%). […] Patients with respiratory symptoms exhibited higher declined lung function measures than those having no respiratory symptoms. High-risk patients with poor prognosis showed more pulmonary function abnormalities. […] Patients with respiratory symptoms had lower values of lung function, including FEV1, FEV1%, FVC, FVC%, DLCO%, and TLC% than those without respiratory symptoms. […] Consequently, presence of respiratory symptom warrants PFTs and echocardiography. Clinicians should intensify their awareness of the association between POEMS syndrome and pulmonary disease to enhance their understanding of the syndrome for improved diagnosis and treatment.

• #46 Pulmonary manifestations of POEM syndrome: a retrospective analysis of 282 cases | BMC Pulmonary Medicine | Full Text

  https://bmcpulmmed.biomedcentral.com/articles/10.1186/s12890-023-02741-9

  Abnormalities in pulmonary manifestations constitute the significant features of POEMS syndrome. Several patients with POEMS syndrome presented with respiratory symptoms at the initial evaluation. These findings underscore the importance of early identification and accurate diagnosis of POEMS syndrome by clinicians, particularly in cases involving lung and multisystem. […] Respiratory symptoms were found in 40.1% of the patients, with dyspnea as the most common symptom (34.4%). […] Patients with respiratory symptoms exhibited higher declined lung function measures than those having no respiratory symptoms. High-risk patients with poor prognosis showed more pulmonary function abnormalities. […] Patients with respiratory symptoms had lower values of lung function, including FEV1, FEV1%, FVC, FVC%, DLCO%, and TLC% than those without respiratory symptoms. […] Consequently, presence of respiratory symptom warrants PFTs and echocardiography. Clinicians should intensify their awareness of the association between POEMS syndrome and pulmonary disease to enhance their understanding of the syndrome for improved diagnosis and treatment.

• #47

  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. […] 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. […] All patients with POEMS syndrome should be assessed or screened for PH by DE, especially those patients with signs of extravascular volume overload. Most PH in POEMS syndrome is reversible or partially reversible after successful treatment. PH is also a negative prognostic factor for survival of POEMS syndrome patients. Active treatments including MDex, ASCT and novel agents may improve outcome of this special group of patients.

• #48

  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. […] 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. […] All patients with POEMS syndrome should be assessed or screened for PH by DE, especially those patients with signs of extravascular volume overload. Most PH in POEMS syndrome is reversible or partially reversible after successful treatment. PH is also a negative prognostic factor for survival of POEMS syndrome patients. Active treatments including MDex, ASCT and novel agents may improve outcome of this special group of patients.

• #49

  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. […] 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. […] All patients with POEMS syndrome should be assessed or screened for PH by DE, especially those patients with signs of extravascular volume overload. Most PH in POEMS syndrome is reversible or partially reversible after successful treatment. PH is also a negative prognostic factor for survival of POEMS syndrome patients. Active treatments including MDex, ASCT and novel agents may improve outcome of this special group of patients.

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

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

  During treatment, hematologic response is monitored by measuring the M-protein concentration and the serum VEGF levels. […] The best way to target peripheral neuropathy is by targeting the clone by anti-plasma cell therapy. […] Neurorehabilitation is an essential part of the treatment plan, and it is essential for sensory as well as motor impairment of the patient. […] The mainstay of treatment is anti-plasma cell therapy such as IMiD or ASCT which will relieve polyneuropathy in most if not all of patients over times. […] The majority of patients will present with signs of fluid overload and anasarca, mainly attributed to the activity of serum VEGF and its contribution to capillary leak. […] Therapy to renal disease specifically is not established, but targeting the clone is the mainstay of treatment with an overall alleviation of signs and symptoms.

• #51 POEMS Syndrome Manifested as Systemic Capillary Leak Syndrome: A Case Report | Antonio | Journal of Medical Cases

  https://www.journalmc.org/index.php/jmc/article/view/1217/665

  POEMS syndrome is a rare paraneoplastic condition secondary to a plasma cell dyscrasia, mainly Castleman Disease. Recognition of a complex combination of its manifestations in a scenario of generalized capillary leak is a huge challenge and at least partially accounts for its poor prognosis. […] The massive pulmonary edema with no evidences whatsoever of heart failure and the anasarca, observed in our patient, is compatible with the diagnosis of SCLS. The arterial hypotension observed could also be explained by this syndrome. Adrenal failure was not evaluated, nevertheless she did not respond to the administration of hydrocortisone.

• #52

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

  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. […] Supportive care considerations include management of endocrinopathy, neuropathy, thrombosis, and infection. […] Effective management requires targeting the underlying plasma cell clone in parallel with proactive management of comorbid risks and POEMS-induced physiological impairments. […] The aim of treatment is to eradicate the underlying plasma cell dyscrasia, but there are limited trial data to guide treatment decisions. […] Clinical nurse specialists or equivalent should act as a keyworker and signpost patients and family members to sources of information and support.

• #53 POEMS syndrome survivor: It’s OK to ask for help | MD Anderson Cancer Center

  https://www.mdanderson.org/cancerwise/multiple-myeloma-poems-syndrome-survivor–its-ok-to-ask-for-help.h00-159223356.html

  One symptom of POEMS syndrome (which stands for polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, skin changes) is leg weakness. […] Most doctors have never even heard of POEMS syndrome, much less treated it. And some neurologists wouldn’t recognize the disease if they saw it. It’s often misdiagnosed as CIDP, so patients with moderate to severe polyneuropathy who are being treated for CIDP should be tested for POEMS. Especially if the CIDP treatments aren’t helping. […] One big lesson I’ve learned through all of this is that it’s OK to ask for help. […] Cancer tears you apart emotionally and physically. It can tear your family apart, too. So, start therapy right away, and if you have children, have them see a therapist as well. […] You think you can handle it. And your spouse may think that, too. But there is so much support out there, from people who really want to help and who know how to. So, turn to them. Just ask. You won’t regret it.

• #54 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 be very serious without treatment. It involves having too many abnormal plasma cells that can damage multiple organs and body systems. […] There isn’t a cure for POEMS syndrome, but treatment can help manage symptoms. Treatments may include: […] Your healthcare provider may also prescribe steroids and diuretics (to relieve swelling) to ease symptoms. […] Effective treatment can stop or slow nerve damage and also help ease other symptoms. Nerve damage may slowly improve over time. […] Contact your provider if you experience any symptoms associated with POEMS syndrome, especially weakness in your arms and legs. […] Once you receive a diagnosis, work closely with your care team to decide which treatments provide the most symptom relief.

• #55 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 be very serious without treatment. It involves having too many abnormal plasma cells that can damage multiple organs and body systems. […] There isn’t a cure for POEMS syndrome, but treatment can help manage symptoms. Treatments may include: […] Your healthcare provider may also prescribe steroids and diuretics (to relieve swelling) to ease symptoms. […] Effective treatment can stop or slow nerve damage and also help ease other symptoms. Nerve damage may slowly improve over time. […] Contact your provider if you experience any symptoms associated with POEMS syndrome, especially weakness in your arms and legs. […] Once you receive a diagnosis, work closely with your care team to decide which treatments provide the most symptom relief.

• #56

  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. […] Provision of in-bed or in-chair exercises, mobility aids, and adaptations have to adjust to the changing needs of the patient. […] The goal of treatment in POEMS syndrome is to suppress the underlying plasma cell clone, but although in some cases it is possible, complete disease suppression (CRH) is not always achievable particularly in frail patients. […] We perform disease evaluation at six months or sooner if there are concerns about an inadequate response. […] Patients with known endocrinopathy should undergo regular clinical and biochemical review of endocrine function, particularly as dysfunction can have major effects on bone health and quality of life.

• #57

  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. […] Provision of in-bed or in-chair exercises, mobility aids, and adaptations have to adjust to the changing needs of the patient. […] The goal of treatment in POEMS syndrome is to suppress the underlying plasma cell clone, but although in some cases it is possible, complete disease suppression (CRH) is not always achievable particularly in frail patients. […] We perform disease evaluation at six months or sooner if there are concerns about an inadequate response. […] Patients with known endocrinopathy should undergo regular clinical and biochemical review of endocrine function, particularly as dysfunction can have major effects on bone health and quality of life.

• #58

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

  POEMS syndrome is a rare, but treatable cause of neuropathy. Further work is required to establish its exact underlying pathophysiology. Current treatment approaches afford good prognosis. Moving forward, randomised-controlled studies, though difficult given the rarity of POEMS syndrome, and the development of prognostic tools, will be important in establishing individualised approaches to patient care.

• #59 POEMS syndrome in Colombia: Clinical findings, therapeutic options and outcomes in a case series | Neurology perspectives

  https://www.elsevier.es/en-revista-neurology-perspectives-17-avance-resumen-poems-syndrome-in-colombia-clinical-S2667049622000515

  POEMS syndrome is a paraneoplastic syndrome associated with a clonal plasma cell neoplasm, in general is a rare disease and its descriptions are based on series and case reports. […] The symptoms, clinical course and treatment of our case series are similar to those published in the global literature. Early diagnosis and active treatment significantly modify the progression of the disease, as well as its overall prognosis. Patients with longer survival and lower disease burden were those who received adjuvant treatment with autologous stem cell transplantation. […] All follow-up patients were treated with combination chemotherapy based on alkylating agents or corticosteroids, cyclophosphamide, thalidomide, lenalidomide with or without autologous stem cell transplantation. Unfortunately, 3 patients died during follow-up in the first 34 years after diagnosis of POEMS syndrome, mainly due to cardiovascular and infectious complications.

• #60 POEMS syndrome in Colombia: Clinical findings, therapeutic options and outcomes in a case series | Neurology perspectives

  https://www.elsevier.es/en-revista-neurology-perspectives-17-avance-resumen-poems-syndrome-in-colombia-clinical-S2667049622000515

  POEMS syndrome is a paraneoplastic syndrome associated with a clonal plasma cell neoplasm, in general is a rare disease and its descriptions are based on series and case reports. […] The symptoms, clinical course and treatment of our case series are similar to those published in the global literature. Early diagnosis and active treatment significantly modify the progression of the disease, as well as its overall prognosis. Patients with longer survival and lower disease burden were those who received adjuvant treatment with autologous stem cell transplantation. […] All follow-up patients were treated with combination chemotherapy based on alkylating agents or corticosteroids, cyclophosphamide, thalidomide, lenalidomide with or without autologous stem cell transplantation. Unfortunately, 3 patients died during follow-up in the first 34 years after diagnosis of POEMS syndrome, mainly due to cardiovascular and infectious complications.

• #61

  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. […] Provision of in-bed or in-chair exercises, mobility aids, and adaptations have to adjust to the changing needs of the patient. […] The goal of treatment in POEMS syndrome is to suppress the underlying plasma cell clone, but although in some cases it is possible, complete disease suppression (CRH) is not always achievable particularly in frail patients. […] We perform disease evaluation at six months or sooner if there are concerns about an inadequate response. […] Patients with known endocrinopathy should undergo regular clinical and biochemical review of endocrine function, particularly as dysfunction can have major effects on bone health and quality of life.

• #62

  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. […] Provision of in-bed or in-chair exercises, mobility aids, and adaptations have to adjust to the changing needs of the patient. […] The goal of treatment in POEMS syndrome is to suppress the underlying plasma cell clone, but although in some cases it is possible, complete disease suppression (CRH) is not always achievable particularly in frail patients. […] We perform disease evaluation at six months or sooner if there are concerns about an inadequate response. […] Patients with known endocrinopathy should undergo regular clinical and biochemical review of endocrine function, particularly as dysfunction can have major effects on bone health and quality of life.

• #63 POEMS Syndrome: Still an Enigma?

  https://www.cancernetwork.com/view/poems-syndrome-still-enigma

  Because of challenges in making the correct diagnosis and the physicians reluctance to administer chemotherapy for a disease characterized by such a low tumoral mass, patients may experience a delay in the initiation of appropriate treatment. […] It is likely that the information provided by Drs. Dispenzieri and Buadi in their article will facilitate earlier diagnosis of POEMS syndrome, to ensure better and earlier treatment. […] The treatment of disease related to toxic monoclonal gammopathies has several aims. First, symptomatic treatments are always critical and may prevent life-threatening complications. Second, in most cases, control of the underlying B-cell lymphoproliferation may improve symptoms of the disease. Third, treatment to address specific complications may be beneficial.

• #64 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 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. […] Autonomic function preservation is typical of this syndrome and distinguishes it from other neuropathies such as diabetic neuropathy. […] 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.

• #65 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 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. […] Autonomic function preservation is typical of this syndrome and distinguishes it from other neuropathies such as diabetic neuropathy. […] 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.

• #66 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. […] Polyneuropathy is one of the two major mandatory criteria for POEMS syndrome diagnosis. In these patients, neurological damage usually follows a demyelinating pattern, with greater axonal lesions and autonomic preservation. However occasionally, atypical cases without axonal lesions and significant autonomic dysfunction are observed. […] This report is significant as this atypical presentation draws attention to the syndromes diagnosis. This report will hopefully stimulate research on the rare POEMS syndrome patients whose polyneuropathy is of unknown etiology and leads to less underreporting.

• #67 How I treat POEMS syndrome

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3425020/

  Papilledema (optic disc edema) is present in at least one-third of patients and may be associated with increased intracranial pressure. […] The neuropathy is usually the dominant characteristic. […] Patients with POEMS syndrome present with a complex conglomerate of symptoms, signs, and objective abnormalities, making the diagnosis, management, and follow-up a challenge. […] Early diagnosis and a prompt multidisciplinary approach increase the likelihood of reduced long-term irreversible morbidity. […] Both therapies directed at other features of the disease as well as emotional support should be a major part of the care plan. […] Serial assessments of clinical stigmata (peripheral neuropathy, volume status, eyes, skin, and organomegaly) of blood (M-spike, VEGF, affected endocrine parameters) should be done every 3 months for at least the first several years.

• #68 NO RHYME BUT WITH REASON: A CASE OF POEMS SYNDROME PRESENTING WITH BILATERAL PAPILLEDEMA – SHM Abstracts | Society of Hospital Medicine

  https://shmabstracts.org/abstract/no-rhyme-but-with-reason-a-case-of-poems-syndrome-presenting-with-bilateral-papilledema/

  POEMS syndrome is an elusive diagnosis as patients can develop seemingly unrelated systemic symptoms at irregular intervals. […] A key feature for our patient was the presence of bilateral papilledema, noted in 26-64% of cases POEMS syndrome. […] Patients with bilateral papilledema and systemic disease; particularly peripheral neuropathy, should be worked up for POEMS syndrome. […] When the pre-test probability of POEMS syndrome is high, a skeletal survey alone cannot confirm the absence of skeletal lesions and a more sensitive study, such as a CT scan, should follow up a negative study in order to rule out bony lesions.

• #69 102  The cost of misdiagnosis in POEMS syndrome | Journal of Neurology, Neurosurgery & Psychiatry

  https://jnnp.bmj.com/content/93/6/A43.2

  POEMS syndrome is a rare cause of neuropathy misdiagnosed as Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP) in 60% of cases. […] VEGF testing should be routine for patients presenting with an acquired demyelinating neuropathy. This will improve early diagnosis of POEMS syndrome, enable effective therapy and reduce costs.

• #70 POEMS syndrome survivor: It’s OK to ask for help | MD Anderson Cancer Center

  https://www.mdanderson.org/cancerwise/multiple-myeloma-poems-syndrome-survivor–its-ok-to-ask-for-help.h00-159223356.html

  One symptom of POEMS syndrome (which stands for polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, skin changes) is leg weakness. […] Most doctors have never even heard of POEMS syndrome, much less treated it. And some neurologists wouldn’t recognize the disease if they saw it. It’s often misdiagnosed as CIDP, so patients with moderate to severe polyneuropathy who are being treated for CIDP should be tested for POEMS. Especially if the CIDP treatments aren’t helping. […] One big lesson I’ve learned through all of this is that it’s OK to ask for help. […] Cancer tears you apart emotionally and physically. It can tear your family apart, too. So, start therapy right away, and if you have children, have them see a therapist as well. […] You think you can handle it. And your spouse may think that, too. But there is so much support out there, from people who really want to help and who know how to. So, turn to them. Just ask. You won’t regret it.

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

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

  Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin abnormalities (POEMS) syndrome is a rare multisystem disease that causes a blood cell disorder and nerve damage, with several other effects. […] There is no cure for POEMS syndrome, but treatments can help alleviate some of the effects. […] Individualized treatments can alleviate specific effects, such as diabetes, thyroid disease, or pain from polyneuropathy. […] Your healthcare team may also prescribe steroids or diuretics (water pills) to ease swelling. […] Without treatment, POEMS syndrome is expected to progress and cause more harm throughout the body. […] Diagnosis and treatment can help your outcome. […] If you or a loved one is living with POEMS syndrome, you must seek medical care from a healthcare team with experience in this condition and learn about maintaining the best possible quality of life.

• #72 102  The cost of misdiagnosis in POEMS syndrome | Journal of Neurology, Neurosurgery & Psychiatry

  https://jnnp.bmj.com/content/93/6/A43.2

  POEMS syndrome is a rare cause of neuropathy misdiagnosed as Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP) in 60% of cases. […] VEGF testing should be routine for patients presenting with an acquired demyelinating neuropathy. This will improve early diagnosis of POEMS syndrome, enable effective therapy and reduce costs.

• #73 POEMS Syndrome | Condition | UAMS Health

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

  POEMS syndrome is a rare, multi-system blood disorder. […] Treatment is aimed at the underlying plasma cell disorder and relieving symptoms. Every patients disease is different. Myeloma Center physicians are dedicated to defining the nuances of each individuals disease and customizing treatment regimens that have the most promise for effective results. […] Patients with POEMS syndrome often need treatment to alleviate the symptoms of neuropathy, extravascular volume overload, and endocrine abnormalities. […] A multi-disciplinary approach to the treatment of POEMS syndrome is essential. The physician team should include myeloma specialists, radiologists, neurologists, nephrologists, cardiologists and other specialists as needed.

• #74 POEMS syndrome | University of Iowa Health Care

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

  POEMS syndrome patients experience numbness, tingling, and weakness in the toes, feet, and later the hands. […] Doctors take a two-pronged approach to treatment. First, they target the M in POEMSthe growth of abnormal plasma cells in the bloodas the underlying cause of the disease. Second, they work to relieve the symptoms caused by the disease. […] Each patients case is different, and doctors will choose a course of treatment that shows the greatest promise for effective results for their individual patient. […] Other treatments to target the abnormal plasma cells may include: Physical therapy to strengthen legs and combat the effects of neuropathy. […] POEMS syndrome patients can be effectively treated and return to an overall normal quality of life. The key for addressing the many facets of POEMS syndrome is being cared for by a team of specialists, including myeloma specialists, radiologists, neurologists, nephrologists, cardiologists, physical therapists, pharmacists, and others.

• #75 POEMS syndrome | University of Iowa Health Care

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

  POEMS syndrome patients experience numbness, tingling, and weakness in the toes, feet, and later the hands. […] Doctors take a two-pronged approach to treatment. First, they target the M in POEMSthe growth of abnormal plasma cells in the bloodas the underlying cause of the disease. Second, they work to relieve the symptoms caused by the disease. […] Each patients case is different, and doctors will choose a course of treatment that shows the greatest promise for effective results for their individual patient. […] Other treatments to target the abnormal plasma cells may include: Physical therapy to strengthen legs and combat the effects of neuropathy. […] POEMS syndrome patients can be effectively treated and return to an overall normal quality of life. The key for addressing the many facets of POEMS syndrome is being cared for by a team of specialists, including myeloma specialists, radiologists, neurologists, nephrologists, cardiologists, physical therapists, pharmacists, and others.

• #76 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/

  He slowly began to regain his strength and his pain started to subside. […] He follows every instruction we give him, says Paba-Prada. […] While most patients with POEMS syndrome respond to treatment, the extent of their recoveries varies widely, Paba-Prada notes. Some recover fully, others dont. Some have residual neuropathy. […] He continues on physical and occupational therapy and tracks his progress meticulously. […] Of the persistence with which hes worked toward recovery, he refers to the strength hes gained from the ordeal, rather than the strength needed to endure it.

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