Materiały źródłowe

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

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

  Disease overview: 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. Diagnoses are often delayed because the syndrome is rare and can be mistaken for other neurologic disorders, most commonly chronic inflammatory demyelinating polyradiculoneuropathy. POEMS syndrome should be distinguished from the Castleman disease variant of POEMS syndrome, which has no clonal PCD and typically little to no peripheral neuropathy but has several of the minor diagnostic criteria for POEMS syndrome. […] 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.

• #1 Diagnosis of POEMS syndrome in a patient with long-standing neuropathy | Neurología (English Edition)

  https://www.elsevier.es/en-revista-neurologia-english-edition–495-articulo-diagnosis-poems-syndrome-in-patient-S2173580818301469

  Diagnosis of POEMS syndrome in a patient with long-standing neuropathy […] Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome is a rare multisystemic disease of paraneoplastic origin, which represents a true clinical challenge. […] The diagnostic criteria are: Major criteria (both mandatory): polyneuropathy (mainly demyelinating) and monoclonal proliferation of plasma cells. Major criteria (at least one must be present): Castleman disease, sclerotic bone lesions, and increased VEGF levels. Minor criteria (at least one must be present): organomegaly (splenomegaly, hepatomegaly, adenopathies); extravascular volume overload (oedemas, pleural oedema, ascites); endocrinopathies (adrenal, thyroid, pituitary, pancreatic); skin changes (hyperpigmentation, hypertrichosis, acrocyanosis, white nails); papilloedema; and thrombocytosis/polycythaemia.

• #1 POEMS Syndrome: Background, Pathophysiology, Etiology

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

  The major criteria are as follows: Polyneuropathy, typically demyelinating (mandatory), Monoclonal plasma cell-proliferative disorder (mandatory), Castleman disease, Sclerotic bone lesions, Vascular endothelial growth factor (VEGF) elevation. […] The minor criteria are as follows: Organomegaly (splenomegaly, hepatomegaly, lymphadenopathy), Extravascular volume overload (edema, pleural effusion, ascites), Endocrinopathy (adrenal, thyroid, pituitary, gonadal, parathyroid, pancreatic), Skin changes (hyperpigmentation, hypertrichosis, glomeruloid hemangiomata, plethora, acrocyanosis, flushing, white nails), Papilledema, Thrombocytosis/polycythemia. […] Classic multiple myeloma has not been associated with the disease. This plasma cell disorder has not been shown to be correlated with the constellation of symptoms noted in patients with POEMS syndrome.

• #1

  https://link.springer.com/article/10.1007/s00415-018-9068-4

  POEMS syndrome is a rare, chronic, disabling paraneoplastic disorder characterized by peripheral neuropathy, organomegaly, endocrinopathy, monoclonal plasma cells disorder and skin changes. Diagnosis relies on the fulfillment of a set of clinical criteria of which polyneuropathy and a monoclonal plasma cell dyscrasia are early and essential features. […] The diagnosis of POEMS syndrome relies primarily on the fulfillment of major and minor criteria. However, the peripheral neuropathy is almost always a constant feature. Indeed, the identification of a chronic progressive, distal, sensorimotor polyneuropathy, along with a monoclonal plasma cell dyscrasia, is essential to the diagnosis of POEMS syndrome. […] The diagnosis of POEMS syndrome is confirmed when both the polyneuropathy and monoclonal gammopathy are present in association with one of the other three major criteria (Castleman disease, sclerotic bone lesions and increased levels of VEGF), and one of the six minor criteria (organomegaly, endocrinopathy, extravascular volume overload, skin changes, papilloedema, thrombocytosis/polycythemia).

• #1

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

  POEMS syndrome has a discernible homogeneity despite its multiplicity of features as the frequent occurrence of so many of the multisystem features together as described in the diagnostic criteria is so recognizable. […] Experienced hematologists, neurologists, radiologists, histopathologists, and neurophysiologists must differentiate POEMS from more common conditions. […] A diagnosis of CIDP that is unresponsive to treatments such as intravenous immunoglobulin or plasma exchange should also trigger a search for POEMS syndrome to avoid delay with further unnecessary and expensive therapies before a diagnosis of is POEMS considered. […] We regard POEMS syndrome as a hematological/neurological emergency and complete diagnostic testing as soon as possible to enable rapid institution of therapy.

• #1 Diagnosis of POEMS syndrome in a patient with long-standing neuropathy | Neurología (English Edition)

  https://www.elsevier.es/en-revista-neurologia-english-edition–495-articulo-diagnosis-poems-syndrome-in-patient-S2173580818301469

  POEMS syndrome is an infrequent entity which falls within the group of plasma cell dyscrasias. Given the rareness, variability, and complexity of forms of onset, diagnosis of POEMS syndrome may be delayed by a median of 13-18 months. […] Therefore, initial clinical suspicion, based on a good history taking and physical examination, is essential to identify which laboratory tests and radiological studies may ultimately enable us to establish a definitive diagnosis. […] Diagnostic delay may entail a decrease in the subsequent response to treatments, increasing sequelae and complications; therefore, one of the main challenges in the context of this disease is to minimise time to diagnosis. […] Clinical suspicion is therefore essential for establishing an early diagnosis. This will provide patients with a specific treatment adapted to their situation, avoiding potential complications and decreasing sequelae, and especially neurological sequelae. Given the greatly variable forms of onset of the syndrome, clinicians should be aware of the multiple manifestations of the disease and perform a directed clinical, analytical, and radiological search.

• #1 Comprehensive Diagnosis and Management of POEMS Syndrome

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

  We regard POEMS syndrome as a hematological/neurological emergency and complete diagnostic testing as soon as possible to enable rapid institution of therapy. […] The objective of the diagnostic work-up is to confirm the diagnosis, establish a biometric baseline and commence treatment promptly. […] A monoclonal plasma cell disorder evidenced as a lambda-restricted plasma cell dyscrasia in the blood, urine, bone marrow, or bone lesions should be sought. […] Serum VEGF levels in POEMS syndrome have a median value of approximately 4000 pg/mL. […] Bone lesions are present in most patients with POEMS and may be sclerotic, lytic, or mixed. […] Nerve conduction studies must be performed to confirm the electrophysiological features of the neuropathy. […] Endocrine abnormalities are a minor criterion for POEMS but crucial to patient morbidity.

• #1 POEMS Syndrome Workup: Laboratory Studies, Imaging Studies, Procedures

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

  When clinical findings are suggestive of POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) syndrome is suggested clinically, a range of laboratory studies may be performed to define the extent of involvement and to establish whether other organ systems are involved. […] Generally, the M protein is immunoglobulin G (IgG)- or immunoglobulin A (IgA)- and small in size (median, 1.1 g/dL). Serum protein immunoelectrophoresis is used to define the nature and the extent of the monoclonal gammopathy. […] For research purposes, cytokine and growth factor levels can be measured. Past studies have found that levels of tumor necrosis factor (TNF)-, interleukin (IL)-6, IL-1, and vascular endothelial growth factor (VEGF) are usually elevated, whereas levels of epidermal growth factor (EGF), fibroblast growth factor (FGF), and platelet-derived growth factor (PDGF) are within the reference range.

• #1 POEMS syndrome-Marrow biopsy

  https://imagebank.hematology.org/imageset/60234/poems-syndromemarrow-biopsy

  The diagnosis of POEMS syndrome can be elusive, with overlap between other medical conditions. It is characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, and skin changes, though not all findings are present in all patients. […] Unique features of bone marrow histopathology in POEMS have previously been described that may carry diagnostic utility. These include: Plasma cell rimming around lymphoid aggregates, Light chain restricted plasma cells, commonly lambda, Megakaryocyte hyperplasia and atypia, Osterosclerosis.

• #1 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. […] 18F-FDG PET/CT is a useful tool for the evaluation of patients with suspected POEMS syndrome. 18F-FDG PET/CT may contribute to the diagnosis, evaluation, and follow-up of patients with POEMS syndrome by providing systematic findings of bone lesions, lymphadenopathy, liver or spleen involvement, serous cavity effusion, and the metabolic status of the lesions. […] Diagnosis of POEMS syndrome is made on the basis of a composite of clinical and laboratory features, which suggests a multisystemic disorder. Among the diagnostic criteria, sclerotic bone lesions, organomegaly, and extravascular volume overload may be detected by imaging modalities.

• #1

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

  Endocrine abnormalities are a minor criterion for POEMS but crucial to patient morbidity. […] The aim of treatment is to eradicate the underlying plasma cell dyscrasia, but there are limited trial data to guide treatment decisions. […] The choice of therapy depends on transplant eligibility. […] The greatest risk is before treatment initiation and during active disease. […] 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. […] An outline of our monitoring approach is summarized in Table 5. Early detection of relapse and institution of therapy are important to avoid accumulation of further disability but relapses are often slow to develop and there is often time to consider what treatment to initiate, and when.

• #1

  https://link.springer.com/article/10.1007/s00415-018-9068-4

  Specific laboratory and recommended strategies for investigations are required to achieve the correct diagnosis and to guide adequate treatment in patients with POEMS syndrome. A thorough clinical assessment, including laboratory tests, neurophysiological and imaging studies, may be required to distinguish POEMS syndrome from other plasma cell dyscrasias and Castleman disease (CD). […] The early diagnosis of POEMS syndrome is critical, as treatment options for the disease in more advanced stages are frequently unsuccessful, despite appropriate therapy being applied. […] The presence of a -type IgA or IgG monoclonal gammopathy is mandatory for the diagnosis of the POEMS syndrome. […] An early and prompt diagnosis is fundamental to provide the best therapeutic intervention and follow-up recommendations.

• #1 POEMS Syndrome | Condition | UAMS Health

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

  A diagnosis of POEMS is determined by the presence of a monoclonal plasma cell disorder, peripheral neuropathy, and one or more of the following: osteosclerotic bone lesions, organomegaly, endocrinopathy, skin changes, increased levels of vascular endothelial growth factor, and swelling. […] A diagnosis of POEMS syndrome begins with a complete medical history and physical exam. […] Laboratory tests of blood and urine are conducted to check protein and hormone levels. Often vascular endothelial growth factor (VEGF) levels are elevated. […] Additional diagnostic testing may include: Skeletal imaging tests, such as CT (computerized tomography) and PET (positron emission tomography) scans, to check for osteosclerotic lesions and enlargement of lymph nodes, liver and spleen; Biopsy of an osteosclerotic lesion and/or bone marrow biopsy to check for abnormal plasma cells; Lymph node biopsy to diagnose co-existent Castleman Disease; Pulmonary function testing; Echocardiogram (ultrasound of the heart); Endocrine evaluation; Nerve conduction studies to evaluate the neuropathy.

• #1 POEMS syndrome: symptoms, diagnosis and treatment – Carenity

  https://www.carenity.us/condition-information/magazine/news/poems-syndrome-everything-there-is-to-know-1817

  POEMS syndrome is frequently confused with chronic inflammatory demyelinating polyneuropathy (CIDP), multiple myeloma, amyloidosis, Guillain-Barr syndrome or MGUS (monoclonal gammopathy of undetermined significance). […] Early diagnosis and response to treatment are essential in reducing morbidity. […] POEMS syndrome is therefore a rare paraneoplastic disorder that can be difficult to diagnose. However, thanks to medical research, progress is being made in understanding its pathophysiology. Combining clinical examination with biological and radiological data enables the diagnosis to be rapidly established and confirmed.

• #1 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. This is a low tumor burden disease. Immunofixation of serum is most commonly required to reveal the presence of a small monoclonal protein in the blood. […] The bone marrow biopsy reveals megakaryocyte hyperplasia and clustering in 54% and 93% of cases, respectively. […] The most common diagnoses in the differential for these patients are CIDP and Guillain-Barr syndrome. The best clues for differentiating POEMS syndrome these diagnoses are: (1) the lack of response to therapies that typically work in these disorders, eg, intravenous gammaglobulin or plasmapheresis; (2) the presence of other features, notably monoclonal protein, thrombocytosis, papilledema, ascites, new endocrine issues, skin changes, or sclerotic bone lesions; (3) the patients sense of feeling unwell; and (4) the presence of clonal plasma cell rimming of lymphoid aggregates found in the bone marrow.

• #1 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. […] There are no established treatment guidelines for POEMS syndrome. Effective management requires targeting the underlying plasma cell clone in parallel with proactive management of comorbid risks and POEMS-induced physiological impairments. Response assessment requires experienced interpretation of multiple clinical and investigation modalities. […] The aim of this framework is to provide clinicians a blueprint for managing POEMS from the moment of diagnostic suspicion through the work-up, selection of therapy, follow-up, and treatment of relapse. We describe the clinical symptoms, diagnostic work-up, treatment, and response assessment.

• #1 Episode 1.4, Part 1 – Comprehensive Diagnosis and Management of POEMS Syndrome – HemaSphere Podcast

  https://hemasphere-podcast.captivate.fm/episode/comprehensive-diagnosis-and-management-of-poems-syndrome-part-1

  What I always say to people is, when we think someone may have POEMS: In our mind, it’s like almost an emergency, a medical emergency, because every week that you delay the diagnosis usually means months of rehab for the patient at the other end. […] The VEGF is usually very high, extremely high. […] In practical terms, it’s very seldom to see a patient at presentation who hasn’t got a VEGF that is exceedingly high. […] POEMS doesn’t have to be POEMS, in terms of all of those features. […] You certainly need enough of them, and in general, you would definitely like to see a „P” and an „M”.

• #1 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 introduction of the VEGF test has contributed to the accurate diagnosis of the disease and the timely assessment of treatment responses. […] Data were extracted from a prospectively maintained database consisting of patients who satisfied the POEMS syndrome diagnostic criteria described by Dispenzieri. […] Misdiagnosis used to be very common in POEMS syndrome. […] However, timelier diagnoses and proper treatments were observed over time, and more patients are diagnosed at an early stage with a lower disease burden. […] Overall survival increased significantly, mainly due to dramatic advances in non-ASCT therapeutic options. […] The increasing availability of drugs targeting plasma cells has changed the management of POEMS syndrome, and it is believed that more advances will be made in the future.

• #1 Episode 1.4, Part 1 – Comprehensive Diagnosis and Management of POEMS Syndrome – HemaSphere Podcast

  https://hemasphere-podcast.captivate.fm/episode/comprehensive-diagnosis-and-management-of-poems-syndrome-part-1

  POEMS syndrome (Polyneuropathy Organomegaly, Endocrinopathy, M-protein and Skin changes) is rare, and systemic manifestations are commonly overlooked initially. Diagnosis is often delayed 12-16 months, during which patients can be severely disabled. […] A recent HemaSphere published article, Comprehensive Diagnosis and Management of POEMS Syndrome, offers a clinical blueprint for managing POEMS, from diagnostics to work-up, therapy selection, follow-up, and relapse treatment. […] One of the interesting features of some of the data we’ve produced is that the average time of diagnosis was about 17 months; which is quite a long time and is now getting shorter. […] The other thing that we discovered was about 50% of patients made their own diagnosis, or at least had suggested it. […] If the person gets to a neurologist — We published a paper that suggested if you have a conduction slowing neuropathy, a demyelinating neuropathy; then you can really rapidly get to a diagnosis by doing a serum immunofixation.

• #1 Comprehensive Diagnosis and Management of POEMS Syndrome

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

  The aim of treatment is to eradicate the underlying plasma cell dyscrasia, but there are limited trial data to guide treatment decisions. […] The choice of therapy depends on transplant eligibility. […] 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.

• #1

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

  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. […] Neurological symptoms have been shown to improve post-ASCT. […] 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.

• #1 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. […] Your care team may recommend you have radiation to destroy abnormal plasma cells if theyre found in limited spots. […] We may use medications (chemotherapy) to slow down the growth of the abnormal plasma cells or to destroy them completely. Your care team will decide which drugs may work for you.

• #1 Episode 1.5, Part 2 – Comprehensive Diagnosis and Management of POEMS Syndrome – HemaSphere Podcast

  https://hemasphere-podcast.captivate.fm/episode/comprehensive-diagnosis-and-management-of-poems-syndrome-part-2

  POEMS syndrome (Polyneuropathy, Organomegaly, Endocrinopathy, M-protein and Skin changes) is rare, and systemic manifestations are commonly overlooked initially. Diagnosis is often delayed 12-16 months, during which patients can be severely disabled. […] A recent HemaSphere published article Comprehensive Diagnosis and Management of POEMS Syndrome offers a clinical blueprint for managing POEMS, from diagnostics to work-up, therapy selection, follow-up, and relapse treatment. […] So that is why we want to diagnose it early and get people well. […] In terms of neuropathy of course, that is another story and that takes time. But that doesn’t really limit their lifespan. […] We often use the phrase of „There is no Amazon Prime in POEMS”. Nothing is going to get better quickly here. But it does get better. So it’s just a matter of pointing the positives out.

• #2

  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. The diagnosis is typically delayed by 1216 months, by which time patients can be severely disabled. […] There are no established treatment guidelines for POEMS syndrome. Effective management requires targeting the underlying plasma cell clone in parallel with proactive management of comorbid risks and POEMS-induced physiological impairments. Response assessment requires experienced interpretation of multiple clinical and investigation modalities. […] The aim of this framework is to provide clinicians a blueprint for managing POEMS from the moment of diagnostic suspicion through the work-up, selection of therapy, follow-up, and treatment of relapse. We describe the clinical symptoms, diagnostic work-up, treatment, and response assessment.

• #2 POEMS syndrome – UpToDate

  https://www.uptodate.com/contents/poems-syndrome/print

  POEMS syndrome (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal plasma cell disorder, Skin changes) is a paraneoplastic process characterized by the presence of a monoclonal plasma cell disorder, peripheral neuropathy, and other features. […] For diagnosis, there are two mandatory criteria – the polyradiculoneuropathy and the plasma cell disorder – and three additional major criteria, one of which must be present, and six minor criteria, one of which must be present (table 1). […] The clinical features, diagnosis, and treatment of POEMS syndrome will be discussed here.

• #2 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. […] Like other syndromes of undefined pathophysiology, POEMS syndrome is diagnosed based on the constellation of symptoms, signs and laboratory findings. […] Patients must have both mandatory criteria and 1 major and 1 minor criteria. […] Mandatory criteria (both): Polyneuropathy, Evidence of monoclonal plasma cell proliferation. […] Major criteria (1): Castleman disease, Elevated level of vascular endothelial growth factor (VEGF), Sclerotic bone lesions. […] Minor criteria (1): Endocrinopathy (other than diabetes and/or hypothyroidism), Extravascular volume overload (eg, ascites, peripheral edema, pleural effusion), Organomegaly, Papilledema, Skin changes, Thrombocytosis or polycythemia.

• #2 POEMS syndrome – Wikipedia

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

  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. […] 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. Clinical examination may show distal wasting, weakness, and sensory impairment affecting both large and small fibre sensory modalities. […] Plasma cell dyscrasias are a group of monoclonal gammopathies in which normal plasma cells in the bone marrow and soft tissues become altered. POEMS syndrome is often associated with an IgA or IgG lambda limited plasma cell dysfunction. […] 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. […] In addition to tests corresponding to the above findings, such as EMG for neuropathy, CT scan, bone marrow biopsy to detect clonal plasma cells, plasma or serum protein electrophoresis to myeloma proteins, other tests can give abnormal results supporting the diagnosis of POEMS syndrome.

• #2 Diagnosis of POEMS syndrome in a patient with long-standing neuropathy | Neurología (English Edition)

  https://www.elsevier.es/en-revista-neurologia-english-edition–495-articulo-diagnosis-poems-syndrome-in-patient-S2173580818301469

  Diagnosis of POEMS syndrome in a patient with long-standing neuropathy […] Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome is a rare multisystemic disease of paraneoplastic origin, which represents a true clinical challenge. […] The diagnostic criteria are: Major criteria (both mandatory): polyneuropathy (mainly demyelinating) and monoclonal proliferation of plasma cells. Major criteria (at least one must be present): Castleman disease, sclerotic bone lesions, and increased VEGF levels. Minor criteria (at least one must be present): organomegaly (splenomegaly, hepatomegaly, adenopathies); extravascular volume overload (oedemas, pleural oedema, ascites); endocrinopathies (adrenal, thyroid, pituitary, pancreatic); skin changes (hyperpigmentation, hypertrichosis, acrocyanosis, white nails); papilloedema; and thrombocytosis/polycythaemia.

• #2 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). […] All of the clinical features are not required for diagnosis. Patients with three of the major criteria necessarily including polyradiculoneuropathy and clonal PCD (plus possibly Castleman, sclerotic bone lesions, VEGF elevation), plus at least one minor criterion can be diagnosed with POEMS. […] Radiographic assessment of bones, elevated VEGF, and bone marrow biopsy aid in confirming the diagnosis. VEGF levels correlate with disease activity.

• #2 A Review of POEMS Syndrome

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

  POEMS syndrome is a rare paraneoplastic syndrome that is caused by an underlying plasma cell disorder. Its main features include polyradiculoneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell disorder, and skin changes. […] The diagnosis of POEMS syndrome is made based on a composite of clinical and laboratory features. The constellation of neuropathy and any of the following should elicit an in-depth patient evaluation for possible POEMS: a lambda-restricted monoclonal protein, thrombocytosis, anasarca, or papilledema. […] Aside from a good clinical history and physical examination, appropriate testing-including radiographic assessment of bones, measurement of VEGF levels, and careful analysis of a bone marrow biopsy-can differentiate this syndrome from other conditions such as chronic inflammatory polyradiculoneuropathy (CIDP), monoclonal gammopathy of undetermined significance (MGUS), neuropathy, and immunoglobulin light chain amyloid neuropathy.

• #2 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. This is a low tumor burden disease. Immunofixation of serum is most commonly required to reveal the presence of a small monoclonal protein in the blood. […] The bone marrow biopsy reveals megakaryocyte hyperplasia and clustering in 54% and 93% of cases, respectively. […] The most common diagnoses in the differential for these patients are CIDP and Guillain-Barr syndrome. The best clues for differentiating POEMS syndrome these diagnoses are: (1) the lack of response to therapies that typically work in these disorders, eg, intravenous gammaglobulin or plasmapheresis; (2) the presence of other features, notably monoclonal protein, thrombocytosis, papilledema, ascites, new endocrine issues, skin changes, or sclerotic bone lesions; (3) the patients sense of feeling unwell; and (4) the presence of clonal plasma cell rimming of lymphoid aggregates found in the bone marrow.

• #2

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

  The objective of the diagnostic work-up is to confirm the diagnosis, establish a biometric baseline and commence treatment promptly. […] A monoclonal plasma cell disorder evidenced as a lambda-restricted plasma cell dyscrasia in the blood, urine, bone marrow, or bone lesions should be sought. […] Serum VEGF levels in POEMS syndrome have a median value of approximately 4000 pg/mL. […] Bone lesions are present in most patients with POEMS and may be sclerotic, lytic, or mixed. […] The presence of sclerotic bone lesions is one of 5 major diagnostic criteria for POEMS. […] We routinely use a sniff nasal inspiratory pressure (SNIP) device to assess inspiratory muscle strength. […] Nerve conduction studies must be performed to confirm the electrophysiological features of the neuropathy.

• #2

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

  POEMS syndrome remains a rare disease and evidence for treatment is largely limited to retrospective cohort studies or case reports. […] Current treatment strategies all target the underlying plasma cell clone, with the exception of bevacizumab, a monoclonal antibody targeting vascular endothelial growth factor (VEGF), which has had disappointing results. […] When POEMS syndrome is suspected, a detailed history and systems examination are essential. Recommended investigations are listed in Table 2. CSF examination usually reveals an elevated protein in the range of 12 g/L but a normal cell count. […] CIDP is an inflammatory, proximal and distal sensory-motor neuropathy, with demyelinating features. Differences between CIDP and POEMS syndrome are listed in Table 3. […] Evidence for treatment in POEMS syndrome is largely limited to retrospective cohort studies, with only one randomised controlled trial (RCT) to date.

• #2 POEMS Syndrome Workup: Laboratory Studies, Imaging Studies, Procedures

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

  Because as many as 10% of patients have marrow involvement with plasma cells, bone marrow biopsy may be indicated. […] Electromyography (EMG) yields findings consistent with polyneuropathy, prominent demyelination, and features of axonal degeneration. […] A study of bone-marrow histology in 87 patients from the Mayo Clinic concluded that the constellation of lambda-restricted monoclonal gammopathy, plasma cell rimming around lymphoid aggregates, and megakaryocytic hyperplasia in bone marrow is highly suggestive of POEMS syndrome, especially in the context of a peripheral neuropathy.

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

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

  Here, according to our research, 18F-FDG PET/CT can visualize many important features of POEMS syndrome, especially the bone lesions. […] The distribution of 18F-FDGavid bone lesions on PET/CT was consistent with other studies using CT or radiographic skeletal survey. […] Thus, when there is a composite of 18F-FDG PET/CT features with bone lesions, lymphadenopathy, liver, or spleen enlargement and hypermetabolic spleen and BM, the differential diagnosis should include POEMS syndrome. However, the predominantly sclerotic change in bone lesions will aid in the diagnosis.

• #2 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-articulo-poems-syndrome-in-colombia-clinical-S2667049622000515

  Regarding the diagnostic criteria proposed by Dispenzieri, these criteria are divided into mandatory, major, and minor criteria. To make the diagnosis of POEMS syndrome at least 1 major and 1 minor criterion is required, always in relation to the mandatory criteria, which are the presence of neuropathy and M protein. […] The 100% of patients had organomegaly, of them 81.2% hepatomegaly, 43.7% splenomegaly, and 56.2% lymphadenopathy. Endocrinopathies was present in 100% of the patients, the most frequent finding of endocrinopathies was hypothyroidism (81.2%). […] 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. […] The results presented in this cohort of patients with POEMS syndrome are consistent with the larger case series published by Bardwick, Takatsuki, Nakanishi, Soubrier, Lin, Wang and Dispenzieri.

• #2 POEMS Syndrome Workup: Laboratory Studies, Imaging Studies, Procedures

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

  When clinical findings are suggestive of POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) syndrome is suggested clinically, a range of laboratory studies may be performed to define the extent of involvement and to establish whether other organ systems are involved. […] Generally, the M protein is immunoglobulin G (IgG)- or immunoglobulin A (IgA)- and small in size (median, 1.1 g/dL). Serum protein immunoelectrophoresis is used to define the nature and the extent of the monoclonal gammopathy. […] For research purposes, cytokine and growth factor levels can be measured. Past studies have found that levels of tumor necrosis factor (TNF)-, interleukin (IL)-6, IL-1, and vascular endothelial growth factor (VEGF) are usually elevated, whereas levels of epidermal growth factor (EGF), fibroblast growth factor (FGF), and platelet-derived growth factor (PDGF) are within the reference range.

• #2 POEMS Syndrome and Multiple Myeloma

  https://www.webmd.com/cancer/multiple-myeloma/what-is-poems-syndrome

  How Is POEMS Syndrome Diagnosed? […] Doctors may use one or several of the following tests to diagnose POEMS syndrome: […] Physical exam: Your doctor will look for signs of POEMS syndrome, such as skin changes or swelling. […] Biopsy: Doctors remove a sample of your bone marrow and examine it for abnormal plasma cells. […] Electromyogram (EMG): An EMG analyzes how well your nerves work. […] Urine or blood tests: These tests can reveal abnormal levels of certain proteins. […] Imaging: X-rays and CT scans help doctors spot bone lesions. […] Other tests: Your doctor may recommend endocrine tests, breathing tests, or echocardiograms.

• #2 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-articulo-poems-syndrome-in-colombia-clinical-S2667049622000515

  The diagnosis is especially challenging, but with an adequate clinical approach, supported by complementary studies focused on findings that identify the systemic involvement of the disease are enough, as well as the search for elements that allow ruling out the main differential diagnoses, such as chronic inflammatory demyelinating polyneuropathy or light chain amyloidosis. To the extent that the diagnosis is made early, patients benefit more from treatment, since it decreases the progression of the disease and has an impact on survival.

• #2 POEMS Syndrome: Clinical Features and Outcomes in a U.K. Cohortlogo-32logo-40logo-60NEJM Journal WatchnejmJW_1L_RGB-b

  https://www.jwatch.org/na52146/2020/08/19/poems-syndrome-clinical-features-and-outcomes-uk-cohort

  A retrospective analysis of cases shows that smooth meningeal thickening provides an additional diagnostic clue differentiating POEMS syndrome from chronic immune demyelinating polyneuropathy. […] Missed or delayed diagnosis is common. […] Mean onset-to-diagnosis time was 15 months. […] Patients diagnosed within 6 months had lower overall neuropathy limitation scores, although more than half of patients were previously misdiagnosed with chronic immune demyelinating polyneuropathy (CIDP). […] POEMS patients were more likely to have meningeal thickening than were patients with CIDP (71% vs. 0%). […] This study highlights the insensitivity of the serum assays used in isolation to diagnose the plasma cell disorder, and it identifies meningeal thickening as another useful diagnostic clue.

• #2 Episode 1.4, Part 1 – Comprehensive Diagnosis and Management of POEMS Syndrome – HemaSphere Podcast

  https://hemasphere-podcast.captivate.fm/episode/comprehensive-diagnosis-and-management-of-poems-syndrome-part-1

  What I always say to people is, when we think someone may have POEMS: In our mind, it’s like almost an emergency, a medical emergency, because every week that you delay the diagnosis usually means months of rehab for the patient at the other end. […] The VEGF is usually very high, extremely high. […] In practical terms, it’s very seldom to see a patient at presentation who hasn’t got a VEGF that is exceedingly high. […] POEMS doesn’t have to be POEMS, in terms of all of those features. […] You certainly need enough of them, and in general, you would definitely like to see a „P” and an „M”.

• #2

  https://link.springer.com/article/10.1007/s00415-018-9068-4

  Specific laboratory and recommended strategies for investigations are required to achieve the correct diagnosis and to guide adequate treatment in patients with POEMS syndrome. A thorough clinical assessment, including laboratory tests, neurophysiological and imaging studies, may be required to distinguish POEMS syndrome from other plasma cell dyscrasias and Castleman disease (CD). […] The early diagnosis of POEMS syndrome is critical, as treatment options for the disease in more advanced stages are frequently unsuccessful, despite appropriate therapy being applied. […] The presence of a -type IgA or IgG monoclonal gammopathy is mandatory for the diagnosis of the POEMS syndrome. […] An early and prompt diagnosis is fundamental to provide the best therapeutic intervention and follow-up recommendations.

• #2 :: 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. […] Reports regarding POEMS syndrome are limited because of its low incidence and the difficulty in diagnosis. […] Therefore, a multidisciplinary approach by hematologists, neurologists, and endocrinologists is essential for accurate and early diagnosis of POEMS syndrome. […] The diagnosis of POEMS syndrome was made according to internationally recognized criteria. […] Elevated VEGF levels are one of the three other major criteria for diagnosing POEMS syndrome and are a useful tool for evaluating disease status and treatment response. […] 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 by the UK group.

• #2 Detection of clonal plasma cells in POEMS syndrome using multiparameter flow cytometry | Scientific Reports

  https://www.nature.com/articles/s41598-024-61034-1

  In this study, we aimed to verify the usefulness of EuroFlow-NGF-based multiparameter flow cytometry (MFC) in POEMS syndrome and explore suitable gating strategies to improve the diagnostic accuracy of POEMS syndrome. […] Our optimized POEMS-flow significantly improved the detection rate of monoclonal PCs (18/25 cases, 72.0%), indicating that POEMS-flow is more suitable for detecting clonal PCs in POEMS syndrome than MM-flow. […] POEMS-flow may be effective in proving PCD, even if it is IFE-negative, and when combined with various examinations, it may improve the diagnostic accuracy of POEMS syndrome. […] This study evaluated the sensitivity of EuroFlow-NGF-based MFC in detecting clonal PCs in POEMS syndrome and demonstrated the usefulness of the optimized POEMS-flow, gating CD38 broadly from dim to bright and CD45 narrowly from negative to dim compared to EuroFlow-NGF-based MFC. This strategy needs to be validated in a larger cohort. This preliminary data indicates that POEMS-flow could improve the identification rate of monoclonal PCs in POEMS syndrome and become a valuable tool for diagnosing POEMS syndrome.

• #2 Episode 1.4, Part 1 – Comprehensive Diagnosis and Management of POEMS Syndrome – HemaSphere Podcast

  https://hemasphere-podcast.captivate.fm/episode/comprehensive-diagnosis-and-management-of-poems-syndrome-part-1

  So that’s a change in perhaps a diagnostic strategy that we need to try to introduce to neurologists – because they can now do a VEGF. […] The message is, for my neurology colleagues and perhaps my hematology colleagues referring to neurology, is: If you’ve got a neuropathy and it’s a conduction slowing neuropathy, then just think „Could this be a bit more complicated than I think it is?” and do those tests. […] I think what Mike and I have found over the years is that quite often POEMS is hidden in plain sight, actually. […] In publishing our paper; our various papers, but most recently the Comprehensive Diagnosis paper, we were hoping to just draw out those features so that people spot them. […] I have to say, as the more we’ve done over the years, the more people do get in touch, and say „Oh, could this be POEMS?”

• #2

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

  Endocrine abnormalities are a minor criterion for POEMS but crucial to patient morbidity. […] The aim of treatment is to eradicate the underlying plasma cell dyscrasia, but there are limited trial data to guide treatment decisions. […] The choice of therapy depends on transplant eligibility. […] The greatest risk is before treatment initiation and during active disease. […] 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. […] An outline of our monitoring approach is summarized in Table 5. Early detection of relapse and institution of therapy are important to avoid accumulation of further disability but relapses are often slow to develop and there is often time to consider what treatment to initiate, and when.

• #2 POEMS syndrome | ABC Medical Center

  https://centromedicoabc.com/en/padecimientos/poems-syndrome/

  It is not easy to diagnose POEMS syndrome because the symptoms can be similar to those of several conditions, so blood tests must be performed to verify hormonal and antibody levels. […] POEMS syndrome, being a chronic condition, cannot be cured, so therapy focuses on controlling the symptoms, delaying the progression of the disease, and preventing serious complications through: […] Chemotherapy. […] Radiation therapy. […] Hematopoietic cell transplant.

• #2 Episode 1.5, Part 2 – Comprehensive Diagnosis and Management of POEMS Syndrome – HemaSphere Podcast

  https://hemasphere-podcast.captivate.fm/episode/comprehensive-diagnosis-and-management-of-poems-syndrome-part-2

  POEMS syndrome (Polyneuropathy, Organomegaly, Endocrinopathy, M-protein and Skin changes) is rare, and systemic manifestations are commonly overlooked initially. Diagnosis is often delayed 12-16 months, during which patients can be severely disabled. […] A recent HemaSphere published article Comprehensive Diagnosis and Management of POEMS Syndrome offers a clinical blueprint for managing POEMS, from diagnostics to work-up, therapy selection, follow-up, and relapse treatment. […] So that is why we want to diagnose it early and get people well. […] In terms of neuropathy of course, that is another story and that takes time. But that doesn’t really limit their lifespan. […] We often use the phrase of „There is no Amazon Prime in POEMS”. Nothing is going to get better quickly here. But it does get better. So it’s just a matter of pointing the positives out.

• #2 Episode 1.5, Part 2 – Comprehensive Diagnosis and Management of POEMS Syndrome – HemaSphere Podcast

  https://hemasphere-podcast.captivate.fm/episode/comprehensive-diagnosis-and-management-of-poems-syndrome-part-2

  First of all, I think from a general looking after the patients point of view, we need a system which enables quick diagnosis. So we need speed with investigations. […] Secondly, we need quick diagnostics. We need more availability of diagnostics. […] We have published in neurology journals as well, to try and highlight neurophysiological features and VEGFs and various things. […] The key message is that it’s so responsive to treatment. […] I think as hematologists we do see people respond a lot to the treatments we give; not necessarily all of them successful. […] My major interest would be: How does that factor get into the nerves? What is it doing to the nerves? Because for me, that’s the way that we intervene in the major disabilities as early as possible. […] What drives it and what the toxicity is that results in the disability.

• #2 POEMS neuropathy: optimising diagnosis and management | Practical Neurology

  https://pn.bmj.com/content/18/4/278

  POEMS syndrome is a rare and disabling autoinflammatory condition characterised by a typical peripheral neuropathy and the presence of a monoclonal plasma cell disorder. The diagnosis of POEMS is a significant challenge because of the heterogeneity of clinical presentations and variation of POEMS features. Patients are often misdiagnosed with another cause of inflammatory neuropathy and receive one or more ineffective immunomodulatory medications, resulting in delayed diagnosis and further clinical deterioration before a diagnosis is made. […] This review draws upon our experience to present the typical features of POEMS syndrome and highlight diagnostic conundrums commonly experienced, supplemented with clinical cases. We provide an investigative guide for clinicians when considering POEMS as the diagnosis, and propose a treatment algorithm that centres on the site and degree of monoclonal cell proliferation.

• #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. […] There are no established treatment guidelines for POEMS syndrome. Effective management requires targeting the underlying plasma cell clone in parallel with proactive management of comorbid risks and POEMS-induced physiological impairments. Response assessment requires experienced interpretation of multiple clinical and investigation modalities. […] The aim of this framework is to provide clinicians a blueprint for managing POEMS from the moment of diagnostic suspicion through the work-up, selection of therapy, follow-up, and treatment of relapse. We describe the clinical symptoms, diagnostic work-up, treatment, and response assessment.

• #3 POEMS Syndrome: Background, Pathophysiology, Etiology

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

  POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) syndrome is a rare multisystemic disease that occurs in the setting of a plasma cell dyscrasia. […] Although no specific case definition has been established for POEMS syndrome, it has generally been agreed that patients with the syndrome should have three or more of the five main features. […] In another approach to establishing the diagnosis, it was proposed that the presence of two major criteria (monoclonal plasma-proliferative disorder and polyneuropathy) along with at least one of seven minor criteria (sclerotic bone lesions, Castleman disease, organomegaly, edema, endocrinopathy, skin changes, and papilledema) is sufficient for diagnosis. […] A refinement of this approach specified that three of five major criteria (the two mandatory ones and one of the three nonmandatory ones) plus at least one of six minor criteria are required.

• #3

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

  The objective of the diagnostic work-up is to confirm the diagnosis, establish a biometric baseline and commence treatment promptly. […] A monoclonal plasma cell disorder evidenced as a lambda-restricted plasma cell dyscrasia in the blood, urine, bone marrow, or bone lesions should be sought. […] Serum VEGF levels in POEMS syndrome have a median value of approximately 4000 pg/mL. […] Bone lesions are present in most patients with POEMS and may be sclerotic, lytic, or mixed. […] The presence of sclerotic bone lesions is one of 5 major diagnostic criteria for POEMS. […] We routinely use a sniff nasal inspiratory pressure (SNIP) device to assess inspiratory muscle strength. […] Nerve conduction studies must be performed to confirm the electrophysiological features of the neuropathy.

• #3 Detection of clonal plasma cells in POEMS syndrome using multiparameter flow cytometry | Scientific Reports

  https://www.nature.com/articles/s41598-024-61034-1

  POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal protein [M-protein], and skin changes) is a rare systemic disorder characterized by various symptoms caused by underlying plasma cell (PC) dyscrasia. Detection of monoclonal PCs is mandatory for the diagnosis of POEMS syndrome; however, the usefulness of EuroFlow-based next-generation flow cytometry (EuroFlow-NGF) in POEMS syndrome for detecting monoclonal PCs in bone marrow (BM) and the gating strategy suitable for flow cytometry study of POEMS syndrome remain unknown. […] The diagnosis is made based on a composite of clinical symptoms and laboratory findings, and the international diagnostic criteria for POEMS syndrome are now extensively used. Polyneuropathy and monoclonal plasma cell-proliferative disorder (PCD, almost always) are included as mandatory major criteria. In most cases of POEMS syndrome, immunofixation electrophoresis (IFE) of serum or urine detects M-protein although other laboratory findings, such as bone marrow (BM) histopathology, next-generation sequencing (NGS), and flow cytometry, are sometimes relevant in proving PCD.

• #3 POEMS Syndrome | Condition | UAMS Health

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

  A diagnosis of POEMS is determined by the presence of a monoclonal plasma cell disorder, peripheral neuropathy, and one or more of the following: osteosclerotic bone lesions, organomegaly, endocrinopathy, skin changes, increased levels of vascular endothelial growth factor, and swelling. […] A diagnosis of POEMS syndrome begins with a complete medical history and physical exam. […] Laboratory tests of blood and urine are conducted to check protein and hormone levels. Often vascular endothelial growth factor (VEGF) levels are elevated. […] Additional diagnostic testing may include: Skeletal imaging tests, such as CT (computerized tomography) and PET (positron emission tomography) scans, to check for osteosclerotic lesions and enlargement of lymph nodes, liver and spleen; Biopsy of an osteosclerotic lesion and/or bone marrow biopsy to check for abnormal plasma cells; Lymph node biopsy to diagnose co-existent Castleman Disease; Pulmonary function testing; Echocardiogram (ultrasound of the heart); Endocrine evaluation; Nerve conduction studies to evaluate the neuropathy.

• #3 Episode 1.4, Part 1 – Comprehensive Diagnosis and Management of POEMS Syndrome – HemaSphere Podcast

  https://hemasphere-podcast.captivate.fm/episode/comprehensive-diagnosis-and-management-of-poems-syndrome-part-1

  What I always say to people is, when we think someone may have POEMS: In our mind, it’s like almost an emergency, a medical emergency, because every week that you delay the diagnosis usually means months of rehab for the patient at the other end. […] The VEGF is usually very high, extremely high. […] In practical terms, it’s very seldom to see a patient at presentation who hasn’t got a VEGF that is exceedingly high. […] POEMS doesn’t have to be POEMS, in terms of all of those features. […] You certainly need enough of them, and in general, you would definitely like to see a „P” and an „M”.

• #3 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. […] Your provider will ask about your medical history and symptoms. Theyll perform a physical exam to check for signs of POEMS syndrome. […] Your provider may perform several tests, including: Blood and urine tests: Your provider may test a blood or urine sample to see if you have high levels of M-protein or VEGF. […] Imaging: X-rays and CT scans allow your provider to see if your bones have hardened or thickened. […] Bone marrow biopsy: A bone marrow biopsy can reveal if you have abnormal-looking plasma cells or a high number of plasma cells. […] Electromyogram (EMG): An EMG is a test that measures nerve function. It can help your provider diagnose polyneuropathy. […] There isnt a cure for POEMS syndrome, but treatment can help manage symptoms. […] Effective treatment can stop or slow nerve damage and also help ease other symptoms.

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

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

  In our study, recent diagnosis year 2014 was associated with a trend for longer PFS in all treated patient groups and also in the ASCT group. […] PET-CT scanning should be routinely performed at diagnosis to detect plasmacytoma or Castleman disease in patients with POEMS syndrome because it is not easy to detect these lesions using bone scintigraphy or plain simple radiography.

• #3

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

  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. […] Neurological symptoms have been shown to improve post-ASCT. […] 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.

• #3 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-articulo-poems-syndrome-in-colombia-clinical-S2667049622000515

  The diagnosis is especially challenging, but with an adequate clinical approach, supported by complementary studies focused on findings that identify the systemic involvement of the disease are enough, as well as the search for elements that allow ruling out the main differential diagnoses, such as chronic inflammatory demyelinating polyneuropathy or light chain amyloidosis. To the extent that the diagnosis is made early, patients benefit more from treatment, since it decreases the progression of the disease and has an impact on survival.

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