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Síndrome POEMS
ORPHA:2905CID-10 · D47.2CID-11 · 2A83.YDOENÇA RARA

A síndrome POEMS é uma síndrome paraneoplásica caracterizada por polirradiculoneuropatia (P), organomegalia (O), endocrinopatia (E), distúrbio clonal de células plasmáticas (M) e alterações cutâneas (S). Outras características incluem papiledema, sobrecarga de volume extravascular, lesões ósseas escleróticas, trombocitose/eritrocitose e níveis elevados de VEGF.

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Introdução

O que você precisa saber de cara

📋

A síndrome POEMS é uma síndrome paraneoplásica caracterizada por polirradiculoneuropatia (P), organomegalia (O), endocrinopatia (E), distúrbio clonal de células plasmáticas (M) e alterações cutâneas (S). Outras características incluem papiledema, sobrecarga de volume extravascular, lesões ósseas escleróticas, trombocitose/eritrocitose e níveis elevados de VEGF.

Pesquisas ativas
5 ensaios
17 total registrados no ClinicalTrials.gov
Publicações científicas
1.373 artigos
Último publicado: 2026 Apr 13

Escala de raridade

CLASSIFICAÇÃO ORPHANET · BRASIL 2024
Unknown
Ultra-rara
<1/50k
Muito rara
1/20k
Rara
1/10k
Pouco freq.
1/5k
Incomum
1/2k
Prevalência
0.0
Europe
Início
Adult
+ elderly
🏥
SUS: Sem cobertura SUSScore: 0%
CID-10: D47.2
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Sinais e sintomas

O que aparece no corpo e com que frequência cada sintoma acontece

Partes do corpo afetadas

📏
Crescimento
6 sintomas
🦴
Ossos e articulações
5 sintomas
🩸
Sangue
5 sintomas
💪
Músculos
3 sintomas
🫃
Digestivo
3 sintomas
🧬
Pele e cabelo
3 sintomas

+ 20 sintomas em outras categorias

Características mais comuns

100%prev.
Polineuropatia
90%prev.
Anormalidade do sistema endócrino
Muito frequente (99-80%)
90%prev.
Aumento do nível circulante de anticorpos
Muito frequente (99-80%)
90%prev.
Doença linfoproliferativa
Muito frequente (99-80%)
90%prev.
Anormalidade da fisiologia da pele
Muito frequente (99-80%)
90%prev.
Hipogonadismo
Muito frequente (99-80%)
51sintomas
Muito frequente (7)
Frequente (33)
Ocasional (11)

Os sintomas variam de pessoa para pessoa. Abaixo estão as 51 características clínicas mais associadas, ordenadas por frequência.

PolineuropatiaPolyneuropathy
Muito frequente100%
Anormalidade do sistema endócrinoAbnormality of the endocrine system
Muito frequente (99-80%)90%
Aumento do nível circulante de anticorposIncreased circulating antibody level
Muito frequente (99-80%)90%
Doença linfoproliferativaLymphoproliferative disorder
Muito frequente (99-80%)90%
Anormalidade da fisiologia da peleAbnormality of skin physiology
Muito frequente (99-80%)90%

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2026
Total histórico1.373PubMed
Últimos 10 anos200publicações
Pico202567 papers
Linha do tempo
2026Hoje · 2026🧪 2009Primeiro ensaio clínico📈 2025Ano de pico
Publicações por ano (últimos 10 anos)

Encontrou um erro ou informação desatualizada? Sugira uma correção →

Genética e causas

O que está alterado no DNA e como passa nas famílias

🧬

Nenhum gene associado encontrado

Os dados genéticos desta condição ainda estão sendo catalogados.

Diagnóstico

Os sinais que médicos procuram e os exames que confirmam

Carregando...

Tratamento e manejo

Remédios, cuidados de apoio e o que precisa acompanhar

Pipeline de tratamentos
Pipeline regulatório — de medicamentos já aprovados a drogas em pesquisa exploratória.
2Fase 26
1Fase 14
·Pré-clínico3
Medicamentos catalogadosEnsaios clínicos· 0 medicamentos · 13 ensaios
Carregando informações de tratamento...

Onde tratar no SUS

Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)

🇧🇷 Atendimento SUS — Síndrome POEMS

🗺️

Selecione um estado ou use sua localização para ver resultados.

Dados de DATASUS/CNES, SBGM, ABNeuro e Ministério da Saúde. Sempre confirme a disponibilidade diretamente com o estabelecimento.

Pesquisa ativa

Ensaios clínicos abertos e novidades científicas recentes

🟢 Recrutando agora

3 pesquisas recrutando participantes. Converse com seu médico sobre a possibilidade de participar.

Outros ensaios clínicos

17 ensaios clínicos encontrados, 5 ativos.

Distribuição por fase
Ver todos no ClinicalTrials.gov
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Publicações mais relevantes

🥉Melhor nível de evidência: Relato de caso
Timeline de publicações
632 papers (10 anos)

Mostrando amostra de 200 publicações de um total de 632

#1

POEMS syndrome: a neuromuscular perspective.

Journal of neurology, neurosurgery, and psychiatry2026 Mar 13

Polyneuropathy, Organomegaly, Endocrinopathy, M-protein and Skin changes (POEMS) syndrome is a rare multisystemic disorder associated with plasma cell dyscrasia, most commonly presenting with peripheral neuropathy. Due to its complex and heterogeneous clinical presentation, misdiagnosis is frequent, particularly with chronic inflammatory demyelinating polyradiculoneuropathy, which often leads to delays in appropriate management. Peripheral nerve involvement in POEMS syndrome is predominantly demyelinating, typically accompanied by early axonal degeneration. Specific clinical, neurophysiological and imaging features are key to differentiating POEMS from other acquired demyelinating neuropathies. Elevated levels of vascular endothelial growth factor (VEGF) play a central role in its pathophysiology, promoting disruption of the blood-nerve barrier and contributing to the systemic manifestations. Accurate diagnosis requires a multidisciplinary approach and is often supported by laboratory testing, including VEGF measurement and monoclonal protein screening as well as advanced imaging techniques. Therapeutic strategies are guided by disease extent and include radiotherapy for localised lesions, systemic chemotherapy and autologous haematopoietic stem cell transplantation for disseminated disease. Early identification and intervention are essential to prevent irreversible nerve damage and optimise long-term functional outcomes. Prompt recognition of characteristic findings not only enables appropriate treatment but may also significantly improve patient prognosis.

#2

Efficacy of radiotherapy and role of adjunctive immunochemotherapy in POEMS syndrome with solitary plasmacytoma.

Blood advances2026 Mar 24

Radiotherapy is a definitive treatment for polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, skin changes (POEMS) syndrome with solitary plasmacytoma. However, data on its efficacy and the role of immunochemotherapy are limited. This study evaluated the treatment outcomes of patients with POEMS syndrome receiving radiotherapy as their primary therapy and compared the results of those treated with adjunctive immunochemotherapy with those without. A retrospective analysis of patients with POEMS syndrome referred to the University College London Hospital from 1998 to 2023 was conducted. Among 150 patients with POEMS syndrome, 30 (20%) presented with solitary plasmacytoma. Sixteen patients (53%) received radiotherapy alone, whereas 14 (47%) had combined treatment with radiotherapy and immunochemotherapy, predominantly lenalidomide, and dexamethasone. Treatment responses were greater in the combined treatment group, with clinical, vascular endothelial growth factor, hematological, and radiological responses of 100%, 92%, 100%, and 100%, respectively, compared with 87%, 92%, 85%, and 79% in patients receiving radiotherapy only. The median follow-up for the entire cohort was 124 months, with a 5-year overall survival rate of 100% and a progression-free survival (PFS) rate of 82%. For those receiving radiotherapy alone, the median follow-up was 124 months, with a 3-year PFS rate of 69%. In contrast, no progression was observed among patients treated with combined therapy during the follow-up period (range, 7-54 months). Six patients experienced disease progression, with all having received radiotherapy alone as their first-line treatment. This study demonstrated the high efficacy of radiotherapy in patients with POEMS syndrome and solitary plasmacytoma and highlighted the potential benefits of combining radiotherapy with immunochemotherapy.

#3

Castleman Disease Variant POEMS Syndrome Presenting as Polyradiculoneuropathy in a Child: A Case Report.

Journal of child neurology2026 Apr

POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, skin changes) is a rare multisystemic disorder linked to plasma cell abnormalities, typically affecting adults. Pediatric cases are extraordinarily rare, with the youngest previously reported patient being 10 years old. We present the case of a 6-year-old boy whose symptoms included progressive lower limb weakness, lymphadenopathy, and thrombocytosis. Initially misdiagnosed as Guillain-Barré syndrome, the finding of lymphadenopathy revealing Castleman disease along with hyperpigmentation, hypertrichosis, papilledema, and hepatomegaly confirmed the diagnosis. Despite the absence of monoclonal protein and sclerotic bone lesions, the multidisciplinary approach led to successful treatment with corticosteroids, thalidomide, and cyclophosphamide, resulting in full remission. This case highlights the diagnostic challenges of POEMS syndrome in children, given its heterogeneity and overlap with other conditions. Early recognition is crucial, as pediatric patients show favorable prognosis with prompt and appropriate treatment.

#4

AESOP syndrome: a rare and early indicator of POEMS syndrome.

Practical neurology2026 Jan 13

Adenopathy and an extensive skin patch overlying a plasmacytoma (AESOP) syndrome is a rare condition characterised by an erythemato-violaceous patch on the skin, accompanied by an underlying solitary plasmacytoma. It may give an early clue for the diagnosis of polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, skin changes (POEMS) syndrome, an uncommon multisystem disorder driven by plasma cell dyscrasia. We describe a 62-year-old man with sensorimotor peripheral neuropathy, unintentional weight loss and a persistent skin rash. Diagnostic findings, including elevated vascular endothelial growth factor, an immunoglobulin A kappa paraprotein and a skin/sternal biopsy, led to the diagnosis of AESOP and POEMS syndrome. This case underscores the importance of early recognition of AESOP syndrome as a potential lifesaving clue for the early diagnosis of POEMS syndrome.

#5

Pachymeningeal thickening and subdural effusion in POEMS syndrome.

Practical neurology2026 Jan 13

Publicações recentes

Ver todas no PubMed

📚 EuropePMC1.091 artigos no totalmostrando 192

2026

Front-line systemic treatment outcomes in POEMS syndrome.

British journal of haematology
2026

When Foot Drop Tells a Bigger Story: POEMS (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal Plasma Cell Disorder, and Skin Changes) Syndrome Revealed by Femoral Plasmacytoma.

Cureus
2026

Atypical Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal Protein, and Skin Changes (POEMS) Syndrome.

Cureus
2026

A woman with fever, feet hypostesia and disseminated papules.

European journal of internal medicine
2026

Monoclonal gammopathies of cutaneous significance: A nomenclature and pathophysiology-based classification.

Journal of the American Academy of Dermatology
2026

Immunophenotypic characteristics of plasma cells in POEMS syndrome.

Cytometry. Part B, Clinical cytometry
2026

POEMS syndrome with cardiovascular lesions as the initial manifestation: a case report and literature review.

Frontiers in cardiovascular medicine
2026

Long-term outcomes of newly diagnosed POEMS syndrome patients treated with bortezomib-based therapies.

Cancer
2026

POEMS syndrome: a neuromuscular perspective.

Journal of neurology, neurosurgery, and psychiatry
2026

Pachymeningeal Involvement in POEMS Syndrome: Longitudinal Follow-Up Study and Correlation With Therapeutic Response.

Journal of the peripheral nervous system : JPNS
2026

Efficacy of radiotherapy and role of adjunctive immunochemotherapy in POEMS syndrome with solitary plasmacytoma.

Blood advances
2025

Case Report: POEMS syndrome secondary to multiple solitary plasmacytomas complicated by hypertriglyceridemia.

Frontiers in medicine
2026

Ivory Vertebra: A Rare Skeletal Manifestation of POEMS Syndrome.

QJM : monthly journal of the Association of Physicians
2026

Neurological Peculiarities of POEMS Syndrome: Experience From a Brazilian University Center.

Muscle &amp; nerve
2025

Refractory ascites: unveiling POEMS syndrome as the underlying cause: a case report and literature review.

Frontiers in medicine
2025

[POEMS syndrome misdiagnosed as systemic sclerosis: A case report].

Beijing da xue xue bao. Yi xue ban = Journal of Peking University. Health sciences
2026

Effective front-line treatment of osteosclerotic myeloma with POEMS syndrome with daratumumab, lenalidomide and dexamethasone: a case report and literature review.

Acta clinica Belgica
2025

Beyond the Obstruction: Recognising Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal Plasma Cell Disorder, and Skin Changes (POEMS) Syndrome in a Patient With Budd-Chiari Syndrome.

Cureus
2025

POEMS syndrome: diagnosis, treatments, and outcomes.

Hematology. American Society of Hematology. Education Program
2025

Characteristics of rare diseases cases: A summary analysis of hospitalized patients at a hospital in Western China from 2015 to 2023.

Intractable &amp; rare diseases research
2025

Case Report: Secondary myelodysplastic syndrome following autologous stem cell transplantation in a patient with POEMS syndrome.

Frontiers in immunology
2025

Multidisciplinary Intensive Care Management of Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal Protein, and Skin Changes (POEMS) Syndrome: A Case Report.

Cureus
2025

Secondary Neuropathy From Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal Gammopathy, and Skin Changes (POEMS) Syndrome: A Case Report.

Cureus
2025

POEMS syndrome complicated by portal hypertension resembling decompensated cirrhosis: a case report and diagnostic insights.

Frontiers in medicine
2025

[POEMS syndrome: A complex diagnostic case under the guise of chronic polyneuropathy].

Zhurnal nevrologii i psikhiatrii imeni S.S. Korsakova
2026

IgA λ-Type Schnitzler Syndrome With Edema and Numbness.

The Journal of dermatology
2025

Lenalidomide-associated pure red cell aplasia during the treatment of POEMS syndrome: A case report and brief review of the literature.

Medicine international
2025

AI-driven proteomics: unlocking early cardiac detection in POEMS syndrome.

Annals of medicine and surgery (2012)
2025

Early diagnosis of POEMS syndrome prompted by skin findings and bone window computed tomography.

Kidney international
2025

[IgM- κ and IgM- λ biclonal POEMS syndrome combined with essential thrombocythemia: a case report].

Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi
2025

Hemangioma as a Primary Manifestation of POEMS Syndrome.

Indian journal of hematology &amp; blood transfusion : an official journal of Indian Society of Hematology and Blood Transfusion
2025

Performance of Large Language Models in Diagnosing Rare Hematologic Diseases and the Impact of Their Diagnostic Outputs on Physicians: Combined Retrospective and Prospective Study.

Journal of medical Internet research
2025

POEMS Syndrome Without a Detectable Monoclonal Peak: The Critical Role of VEGF and Bone Marrow Biopsy in Diagnosis.

Case reports in hematology
2026

Castleman Disease Variant POEMS Syndrome Presenting as Polyradiculoneuropathy in a Child: A Case Report.

Journal of child neurology
2025

Description of TEMPI syndrome in Waldenström's macroglobulinemia.

Klinicka onkologie : casopis Ceske a Slovenske onkologicke spolecnosti
2025

Targeting CD38 with Daratumumab in a Newly Diagnosed Case of POEMS Syndrome with a 17p Deletion in a Solitary Plasmacytoma of the Bone: A Case Study and Literature Review.

Neurology India
2025

POEMS Syndrome Masquerading as Metastatic Prostate Cancer Based on PSMA Avid Lesions.

Case reports in hematology
2025

Concurrent AESOP and POEM syndrome presenting as an indurated plaque: AESOP's paraneoplastic POEM.

JAAD case reports
2025

POEMS syndrome: Two cases for the general physician.

Clinical medicine (London, England)
2026

AESOP syndrome: a rare and early indicator of POEMS syndrome.

Practical neurology
2025

POEMS syndrome presenting with Vibrio vulnificus-like cutaneous lesions: a CARE guidelines-compliant case report.

Frontiers in medicine
2025

The morphological spectrum of Castleman disease and related disorders: a report from the Lymphoma Workshop of the 22nd Meeting of the European Association of Hematopathology.

Virchows Archiv : an international journal of pathology
2025

Interleukin-6 is a highly prognostic biomarker for POEMS syndrome.

Leukemia
2025

Unicentric Castleman disease following POEMS syndrome remission.

Journal of hematopathology
2025

Delayed Diagnosis of Poems Syndrome, How AI Tools Can Enhance Diagnostic Accuracy.

European journal of case reports in internal medicine
2025

[Chinese expert consensus on the diagnosis and treatment of POEMS syndrome (2025)].

Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi
2025

Vasculitic Neuropathy With Iron Deficiency-Related Chronic Inflammation Followed by Elevated Serum Vascular Endothelial Growth Factor: A POEMS (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal Gammopathy, and Skin Changes) Syndrome Mimic.

Cureus
2025

Corrigendum to "First-line therapy with daratumumab, lenalidomide and dexamethasone for patient with POEMS syndrome: A case report" [Leukemia Research Reports (2024) Volume 22, 100491].

Leukemia research reports
2025

A scoping review: POEMS syndrome (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal plasma cell disorder and Skin Changes), physical recovery, and rehabilitation.

Clinical rehabilitation
2025

Teclistamab for heavily pretreated relapsed / refractory POEMS syndrome.

Haematologica
2025

Essential thrombocythemia as an initial presentation of polyneuropathy, organomegaly, endocrinopathy, M-protein, skin changes syndrome with complete response to the bortezomib, cyclophosphamide, dexamethasone regimen: a case report.

Journal of medical case reports
2025

A case report of Castleman disease variant of POEMS syndrome presenting with prominent polyserositis and renal impairment.

Frontiers in medicine
2026

Pachymeningeal thickening and subdural effusion in POEMS syndrome.

Practical neurology
2025

Update on paraproteinemic neuropathy.

Current neurology and neuroscience reports
2025

Successful treatment with carfilzomib and dexamethasone for relapsed/refractory POEMS syndrome: a case report and review of literature.

Frontiers in oncology
2025

[A case of POEMS syndrome presenting craniocervical vascular stenosis].

Rinsho shinkeigaku = Clinical neurology
2025

Daratumumab (anti-CD38)- and elotuzumab (anti-SLAMF7)-based treatments for refractory POEMS syndrome: a single-center case series.

Hematology (Amsterdam, Netherlands)
2025

Lecture 4. Clonal Plasma Cell Precursors.

Hematological oncology
2025

Growth Differentiation Factor-15 as a Potential Biomarker for Renal Involvement in POEMS Syndrome.

International journal of nephrology and renovascular disease
2025

IgA nephropathy, non-cirrhotic portal fibrosis, and POEMS syndrome: A rare combination in the long-term follow-up of Sjögren's syndrome.

The Journal of international medical research
2025

Unmasking POEMS (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal Gammopathy, and Skin Changes) Syndrome in a Heart Failure Patient: A Diagnostic Challenge.

Cureus
2025

Successful assisted pregnancy outcome in a patient with POEMS syndrome.

BMJ case reports
2025

Plasma cell myeloma with an evolving diagnostic complexity: A case report.

Oncology letters
2025

Clonal Hematopoietic Mutations in Plasma Cell Disorders: Clinical Subgroups and Shared Pathogenesis.

Genomics, proteomics &amp; bioinformatics
2025

Diagnostic Challenges of Polyneuropathy, Organomegaly, Endocrinopathy, M-protein, and Skin Changes (POEMS) Syndrome: A Rare Case Report and Review of the Literature.

Cardiovascular &amp; hematological disorders drug targets
2025

Idiopathic Nodular Glomerulosclerosis in a Long-term Passive Smoker With Recently Diagnosed Hypertension: A Case Report.

Journal of investigative medicine high impact case reports
2025

VEGF as a Relapse Marker and an Intensity of Disease Marker in Patients Affected by POEMS Syndrome Treated With Autologous Peripheral Blood Stem Cell Transplantation.

Hematological oncology
2025

Prevalence and Spectrum of Neuropathies in a Cohort of 585 Patients With Immunoglobulin A Monoclonal Gammopathy.

European journal of neurology
2025

Clinical and biochemical characteristics for patients with polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes syndrome: a pilot observational study.

Frontiers in neurology
2025

Daratumumab desensitization: Novel approaches in POEMS syndrome experience.

Journal of oncology pharmacy practice : official publication of the International Society of Oncology Pharmacy Practitioners
2025

Association of co-existing vitamin B6 and B12 deficiency with polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes (POEMS) syndrome: a case report.

BMC neurology
2025

POEMS Syndrome Presenting as First-Time Heart Failure Exacerbation.

JACC. Case reports
2024

Unraveling Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal Gammopathy, and Skin Changes Syndrome: Diagnostic Challenges and Therapeutic Strategies at a National Tertiary Care Center.

Cureus
2025

Clinical Impact of Osteolytic Bone Lesions in POEMS Syndrome: A Single-Center Experience of 114 Patients.

Hematological oncology
2025

Utility of word embeddings from large language models in medical diagnosis.

Journal of the American Medical Informatics Association : JAMIA
2024

Case report: Atypical POEMS syndrome without polyneuropathy, complicated by borderline TAFRO syndrome.

Frontiers in medicine
2024

A case report of an M protein-negative patient with POEMS syndrome associated with renal involvement.

BMC nephrology
2024

Castleman disease variant of POEMS syndrome without M protein: a case report.

Frontiers in oncology
2024

First-line therapy with daratumumab, lenalidomide and dexamethasone for patient with POEMS syndrome: A case report.

Leukemia research reports
2025

Capillary leak phenotype as a major cause of death in patients with POEMS syndrome.

Leukemia
2025

Castleman disease-type histopathological patterns of lymph nodes in patients with plasma cell neoplasia and POEMS syndrome.

Annals of diagnostic pathology
2024

POEMS syndrome complicated with acute cerebral infarction: A case report and literature review.

Asian journal of surgery
2025

Polyneuropathy With Motor Conduction Block in POEMS.

Muscle &amp; nerve
2025

Osteosclerosing multiple myeloma: A case highlighting critical value of image findings for the diagnosis.

Radiology case reports
2024

[POEMS syndrome. Report of three cases and literature review].

Revista medica del Instituto Mexicano del Seguro Social
2024

[POEMS syndrome mimicking CIDP].

Der Nervenarzt
2024

Disease Characteristics and Outcomes of 493 Young Myeloma Patients Treated With Modern Therapies: A Canadian Myeloma Research Group Database Study.

Cancer medicine
2024

[POEMS Syndrome: Report of Two Cases].

Revista medica de Chile
2025

Top 10 Clinical Pearls in Paraproteinemic Neuropathies.

Seminars in neurology
2024

Castleman Disease: A Rare Lymphoproliferative Disorder With Diverse Clinical Presentation, Diagnosis, and Treatment Approach.

Cureus
2024

Efficacy of nebulized GM-CSF inhalation in preventing oral mucositis in patients undergoing hematopoietic stem cell transplantation: A retrospective study.

Heliyon
2024

Acute leukoencephalopathy associated with daratumumab treatment in POEMS syndrome: a case report.

Frontiers in immunology
2024

[Diagnosis and management of POEMS syndrome].

[Rinsho ketsueki] The Japanese journal of clinical hematology
2024

A case of paraprotein-negative POEMS syndrome: Case report and literature review.

Medicine
2024

[Clinical characteristics of POEMS syndrome initially diagnosed as increased extravascular volume load: a single-center retrospective study].

Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi
2024

Intersecting Pathologies: A Case Report on the Co-presentation of Quasi-Moyamoya Disease and Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal Plasma Cell Disorder, and Skin Changes (POEMS) Syndrome.

Cureus
2025

Atypical POEMS syndrome: Report of a patient.

Medicina clinica
2024

Dramatically elevated plasma vascular endothelial growth factor levels from influenza A infection in polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes syndrome: A case report.

EJHaem
2024

Case report: DKRd regimen in the treatment of newly diagnosed POEMS syndrome and literature review.

Frontiers in oncology
2025

The Latin-American Experience in POEMS Syndrome: A Study of the GELAMM (Grupo de Estudio Latinoamericano de Mieloma Múltiple).

Acta haematologica
2024

Case report: POEMS syndrome with portal hypertension.

Frontiers in medicine
2025

Requirement of Repeated Serum VEGF Measurements in POEMS Syndrome.

Internal medicine (Tokyo, Japan)
2024

Plasma cell and neutrophil enriched neovascularization with granulomatous lymphangitis in POEMS syndrome.

JAAD case reports
2024

Ixazomib in POEMS syndrome: 'Ixa'ctly what we need?

British journal of haematology
2024

Monoclonal gammopathy of clinical signifi cance with osteosclerotic lesions - a case report and a literature review.

Klinicka onkologie : casopis Ceske a Slovenske onkologicke spolecnosti
2024

An open-label, prospective trial to evaluate the efficacy and safety of ixazomib in combination with cyclophosphamide and dexamethasone in patients with newly diagnosed POEMS syndrome.

British journal of haematology
2024

[The Preventive Effect of Levofloxacin Combined with G-CSF or Only G-CSF Supportive Therapy on Infection in Autologous Hematopoietic Stem Cell Transplantation].

Zhongguo shi yan xue ye xue za zhi
2024

Unusual Types of Plasma Cell Dyscrasias Associated With HIV: Polyneuropathy, Organomegaly, Endocrinopathy, M-protein, and Skin Changes (POEMS) Syndrome.

Cureus
2024

Long-term outcomes of newly diagnosed POEMS syndrome patients who received first-line lenalidomide-based therapy.

Haematologica
2024

Recent insights into haematology and peripheral nerve disease.

Current opinion in neurology
2024

POEMS syndrome with undetectable M-protein: a case report and literature review.

Diagnostic pathology
2024

[A race against time: POEMS syndrome].

Ideggyogyaszati szemle
2024

Paraproteinemic neuropathies.

Muscle &amp; nerve
2024

TAFRO Syndrome on 18F-FDG-PET/CT: An Appealing Diagnostic Tool.

Diagnostics (Basel, Switzerland)
2024

Elevated serum levels of soluble B-cell maturation antigen as a prognostic biomarker for multiple myeloma.

Clinical and experimental immunology
2024

Detection of clonal plasma cells in POEMS syndrome using multiparameter flow cytometry.

Scientific reports
2024

POEMS syndrome associated with IgM lambda paraprotein. A case report and review of the literature.

Journal of the neurological sciences
2024

Whole-body magnetic resonance neurography in patients with chronic inflammatory demyelinating polyneuropathy.

Muscle &amp; nerve
2024

Hydrocephalus associated with POEMS syndrome.

Medicina clinica
2025

POEMS syndrome-An unusual cause for high-gradient ascites.

Indian journal of gastroenterology : official journal of the Indian Society of Gastroenterology
2024

A Case Report of Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal Gammopathy, and Skin Changes (POEMS) Syndrome: A Diagnostic Iceberg.

Cureus
2024

The challenge of detecting monoclonal protein in POEMS syndrome: two case reports.

Hong Kong medical journal = Xianggang yi xue za zhi
2024

Gammopathic dermopathy: characterization of cutaneous MGUS.

Leukemia &amp; lymphoma
2024

Non-POEMS osteosclerotic multiple myeloma: Clinical characteristics and differential diagnosis.

Journal of bone oncology
2024

Hematologic malignancies and hematopoietic stem cell transplantation.

Handbook of clinical neurology
2024

Adenopathy and Extensive Skin patch Overlaying a Plasmacytoma and other proliferation (AESOP) syndrome associated with Hodgkin lymphoma.

Annales de dermatologie et de venereologie
2024

Low serum total cholesterol levels predict inferior prognosis of patients with POEMS syndrome.

Discover oncology
2024

Intramedullary leukocytoclastic vasculitis and neutrophil extracellular trap (NET) formation in POEMS syndrome.

Annals of hematology
2024

Calciphylaxis in POEMS syndrome: Case report.

Rare (Amsterdam, Netherlands)
2024

A case of POEMS syndrome comprising of a complicated diagnostic procedure: Case report.

Medicine
2023

Chronic Inflammatory Demyelinating Polyradiculoneuropathy as a Presentation of Plasmacytoma.

Indian journal of nuclear medicine : IJNM : the official journal of the Society of Nuclear Medicine, India
2023

Case report: POEMS syndrome masquerades as diabetic foot.

Frontiers in neurology
2024

Polyneuropathy, organomegaly, endocrinopathy, M-protein, skin changes syndrome with dilated cardiomyopathy: A case report.

World journal of clinical cases
2024

Efficacy of bortezomib, cyclophosphamide, and dexamethasone for newly diagnosed POEMS syndrome patients.

Therapeutic advances in neurological disorders
2024

From hepatomegaly to POEMS syndrome: A case report.

Hepatology forum
2024

A Multicenter Analysis of Clinical Features and Long-Term Outcomes of POEMS Syndrome in Korea.

Journal of Korean medical science
2023

Clinico-pathological profile of patients with plasma cell neoplasms with special reference to bone marrow fibrosis and amyloid deposition.

Journal of clinical and experimental hematopathology : JCEH
2023

POEMS (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal Plasma Cell Disorder, and Skin Changes) Syndrome as a Sequela of Castleman Disease: A Case Report.

Cureus
2023

POEMS syndrome characterized by bone lesions: A case report.

Medicine
2024

Specific dermoscopic findings in glomeruloid haemangioma for diagnosis of POEMS syndrome.

Journal of the European Academy of Dermatology and Venereology : JEADV
2023

Pulmonary manifestations of POEM syndrome: a retrospective analysis of 282 cases.

BMC pulmonary medicine
2023

Unraveling the Neurological Complexity of Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal Protein, and Skin Changes Syndrome: A Report of a Challenging Case of a Young Woman and Cutting-Edge Advancements in the Field.

Diseases (Basel, Switzerland)
2023

Concomitant AESOP and POEMS Syndrome in the Context of Osseous and Cutaneous Plasmacytomas: Answer.

The American Journal of dermatopathology
2024

Long-Term Outcomes of Autologous Stem Cell Transplantation in Patients with Newly Diagnosed POEMS Syndrome.

Transplantation and cellular therapy
2024

Giant papillary hemangioma-A rare tumor with an exceptional size.

Journal of cutaneous pathology
2023

POEMS syndrome in the 21st century: A bibliometric analysis.

Heliyon
2023

Small vessel platelet thrombosis in the peripheral nerves in POEMS syndrome.

Muscle &amp; nerve
2023

A case report on POEMS syndrome with high parathyroid hormone and repeated surgery, a rare presentation of a rare disease.

International journal of surgery case reports
2023

Castleman Disease Variant of Poems Syndrome Complicated with Multiple Osteosclerotic Lesions.

Indian journal of hematology &amp; blood transfusion : an official journal of Indian Society of Hematology and Blood Transfusion
2023

Baricitinib treatment for refractory skin changes in POEMS syndrome: a case report.

Frontiers in pharmacology
2024

Adenopathy and extensive skin patch overlying a plasmacytoma (AESOP) syndrome: a case report and literature review.

Annals of hematology
2023

POEMS syndrome: Update on diagnosis, risk-stratification, and management.

American journal of hematology
2024

A Rare Cause of Chronic Diarrhea and Peripheral Neuropathy.

Gastroenterology
2024

Regular assessment of serum vascular endothelial growth factor levels to monitor POEMS syndrome.

Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology
2023

POEMS syndrome: an unusual cause of exuberant callus formation.

BMJ case reports
2024

What is the best treatment strategy before autologous peripheral blood stem cell transplantation in POEMS syndrome?

Haematologica
2023

Characteristics and outcome of infectious complications after autologous hematopoietic cell transplantation in multiple myeloma patients.

Transplant infectious disease : an official journal of the Transplantation Society
2023

Daratumumab monotherapy for relapsed POEMS syndrome.

American journal of hematology
2023

[Identification of transcriptional features and surface markers of plasma cell clones in POEMS syndrome by single-cell RNA sequencing].

[Rinsho ketsueki] The Japanese journal of clinical hematology
2023

POEMS syndrome: A rare entity.

Indian journal of pathology &amp; microbiology
2023

Treatment-responsive glycogen storage myopathy in a patient with POEMS syndrome: A new monoclonal gammopathy-associated myopathy.

European journal of neurology
2023

Treatment and outcome of second-line therapy for POEMS syndrome after autologous stem cell transplantation.

Bone marrow transplantation
2023

Alterations in choroidal vascular structures due to serum levels of vascular endothelial growth factor in patients with POEMS syndrome.

Scientific reports
2023

Ultrasound-based radiomic analysis of the peripheral nerves for differentiation between CIDP and POEMS syndrome.

Acta radiologica (Stockholm, Sweden : 1987)
2023

A case report of POEMS syndrome with lower limb numbness and weakness.

Asian journal of surgery
2023

Daratumumab, carfilzomib, and pomalidomide for the treatment of POEMS syndrome: The Mayo Clinic Experience.

Blood cancer journal
2023

POEMS Syndrome-Clinical Picture and Management. Current Knowledge.

Clinical lymphoma, myeloma &amp; leukemia
2023

Dutcher bodies as a diagnostic key to POEMS syndrome.

EJHaem
2023

T-Cells Subsets in Castleman Disease: Analysis of 28 Cases Including Unicentric, Multicentric and HHV8-Related Clinical Forms.

International journal of molecular sciences
2023

Porto-sinusoidal vascular disorder, report of a novel association with POEMS syndrome. Future challenge for the hepatologist.

Clinics and research in hepatology and gastroenterology
2023

Epidemiology of monoclonal gammopathy in Morocco - A hospital-based study.

Cancer reports (Hoboken, N.J.)
2023

Surgical revascularization for quasi-moyamoya disease associated with polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome: a case report and literature review.

Nagoya journal of medical science
2023

POEMS syndrome: origination from clonal plasma cells or B cells?

Hematology (Amsterdam, Netherlands)
2023

[POEMS syndrome in the elderly. Clinical case and review of the literature].

Revista espanola de geriatria y gerontologia
2023

Successful treatment with bortezomib for POEMS syndrome, overcoming complicated severe heart block.

Clinical case reports
2023

[POEMS Syndrome with Peripheral Edema and Ascites as the Main Manifestations:Report of One Case].

Zhongguo yi xue ke xue yuan xue bao. Acta Academiae Medicinae Sinicae
2023

Biclonal lymphoplasmacytic lymphoma/Waldenström macroglobulinemia associated with POEMS syndrome: A case report and literature review.

Oncology letters
2022

POEMS Syndrome - A Diagnostic Dilemma with Challenging Presentation.

Journal of community hospital internal medicine perspectives
2023

Ocular complications of plasma cell dyscrasias.

European journal of ophthalmology
2023

The exploration of B cell maturation antigen expression in plasma cell dyscrasias beyond multiple myeloma.

BMC cancer
2023

Coexistence of primary mediastinal MALT lymphoma and multiple myeloma like POEMS syndrome: A case report and literature review.

Medicine
2023

Polyneuropathy organomegaly endocrinopathy M-protein and skin changes syndrome with ascites as an early-stage manifestation: A case report.

World journal of clinical cases
2022

Solitary hyoid plasmacytoma with unicentric Castleman disease: A case report and review of literature.

World journal of clinical cases
2023

POEMS syndrome presents with a distended abdomen: A case report.

The Journal of international medical research
2022

Ixazomib Combined With Autologous Stem Cell Transplantation for POEMS Syndrome: A Case Report and Meta-Analysis.

Technology in cancer research &amp; treatment
2022

Better prognosis in POEMS patients with cerebral infarction before polyneuropathy.

Heliyon
2022

A rare case of POEMS syndrome presenting as essential thrombocythemia.

Oxford medical case reports
2022

POEMS (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal Gammopathy, and Skin Changes) Syndrome Masquerading as Chronic Inflammatory Polyradiculoneuropathy: A Case Report.

Cureus
2022

Recent Advances in the Treatment and Supportive Care of POEMS Syndrome.

Journal of clinical medicine
2022

A POEMS syndrome patient with idiopathic non-cirrhotic portal hypertension received the transjugular intrahepatic portosystemic shunt: a case report and literature review.

Nigerian journal of clinical practice
2022

POEMS syndrome: A rare cause of ascites and pelvic effusion.

Clinical case reports
2023

Mutations in the plasma cell clone identify mechanism of polyneuropathy in a case of POEMS syndrome associated with Castleman disease and multiple myeloma.

Annals of hematology
Ver todos os 1.091 no EuropePMC

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Comunidades

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Doenças relacionadas

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Referências e fontes

Bases de dados externas citadas neste artigo

Publicações científicas

Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.

  1. POEMS syndrome: a neuromuscular perspective.
    Journal of neurology, neurosurgery, and psychiatry· 2026· PMID 41617534mais citado
  2. Efficacy of radiotherapy and role of adjunctive immunochemotherapy in POEMS syndrome with solitary plasmacytoma.
    Blood advances· 2026· PMID 41569791mais citado
  3. Castleman Disease Variant POEMS Syndrome Presenting as Polyradiculoneuropathy in a Child: A Case Report.
    Journal of child neurology· 2026· PMID 41004647mais citado
  4. AESOP syndrome: a rare and early indicator of POEMS syndrome.
    Practical neurology· 2026· PMID 40813066mais citado
  5. Pachymeningeal thickening and subdural effusion in POEMS syndrome.
    Practical neurology· 2026· PMID 40555482mais citado
  6. Morphological characteristics of the renal lesion in TAFRO syndrome and POEMS syndrome: a retrospective and multicenter study.
    Clin Exp Nephrol· 2026· PMID 41975025recente
  7. POEMS syndrome presenting as Porto-sinusoidal vascular disease: A case report.
    Clin Res Hepatol Gastroenterol· 2026· PMID 41966479recente
  8. Clinical impact and prognostic implications of concurrent amyloid deposition in patients with POEMS syndrome: A single-center retrospective study.
    Ann Hematol· 2026· PMID 41954675recente
  9. A rare case of POEMS syndrome with unique endoscopic presentation.
    Ann Hematol· 2026· PMID 41954634recente
  10. Prognostic value of the neutrophil-to-lymphocyte ratio in POEMS syndrome.
    Clin Exp Med· 2026· PMID 41925928recente

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:2905(Orphanet)
  2. MONDO:0017364(MONDO)
  3. GARD:7411(GARD (NIH))
  4. Busca completa no PubMed(PubMed)
  5. Q2553422(Wikidata)

Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.

Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar

Síndrome POEMS
Compêndio · Raras BR

Síndrome POEMS

ORPHA:2905 · MONDO:0017364
Prevalência
Unknown
Herança
Unknown
CID-10
D47.2 · Gamopatia monoclonal
CID-11
Ensaios
5 ativos
Início
Adult, Elderly
Prevalência
0.0 (Europe)
MedGen
UMLS
C0085404
EuropePMC
Wikidata
Papers 10a
Evidência
🥉 Relato de caso
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