POEMS综合征

定义 英文原文（暂无中文）

POEMS syndrome is a paraneoplastic syndrome characterized by polyradiculoneuropathy (P), organomegaly (O), endocrinopathy (E), clonal plasma cell disorder (M), and skin changes (S). Other features include papilledema, extravascular volume overload, sclerotic bone lesions, thrombocytosis/erythrocytosis, and elevated VEGF levels.

别名

多发性神经病-内分泌病-浆细胞恶液质综合征

基本事实

遗传方式

未知

发病年龄

成年期、老年期

患病率

1-9 / 1 000 000（Japan）

临床表型 51

近两年的全球研究 346L2

2024/08 起在 Europe PMC 检索所得，按发表时间倒序显示最近 20 篇。标题未译成中文——自动翻译需要接入 LLM 服务，尚未引入。

• 2026-09综述开放获取
  A Quarter Century of EHD Protein Research: From Endosomal Recycling to Ciliopathies
  Traffic (Copenhagen, Denmark) · DOI · Europe PMC
• 2026-08
  Clinicopathological features and outcomes of glomerular microangiopathy: a retrospective cohort study
  Renal failure · DOI · Europe PMC
• 2026-08
  Monoclonal gammopathy of clinical significance: a guide for nephrologists
  Nephrology, dialysis, transplantation : official publication of the Eu · DOI · Europe PMC
• 2026-07
  Autologous stem cell transplantation in POEMS syndrome: Multicenter real-world treatment patterns and outcomes
  Bone marrow transplantation · DOI · Europe PMC
• 2026-07病例报告
  HIV-negative KSHV/HHV8-associated multicentric Castleman disease with concurrent Kaposi sarcoma and POEMS-like manifestations
  Journal of hematopathology · DOI · Europe PMC
• 2026-07
  Acquired vitamin B6 deficiency in POEMS: a marker of disease activity and cause of neuropathy?
  Journal of neurology, neurosurgery, and psychiatry · DOI · Europe PMC
• 2026-07
  Detection of M-protein by iMS-IP and iMS-FLC assays redefines previously non-secretory POEMS syndrome
  Clinical chemistry and laboratory medicine · DOI · Europe PMC
• 2026-07
  POEMS Syndrome: 2026 Update on Diagnosis, Risk-Stratification, and Management
  American journal of hematology · DOI · Europe PMC
• 2026-07综述
  Lambda restricted light chain as pathogenic cause of POEMS syndrome: a review of the hypothesis
  Frontiers in immunology · DOI · Europe PMC
• 2026-07
  Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal Plasma Cell Disorder, and Skin Changes (POEMS) Syndrome Presenting after Immune Thrombocytopenic Purpura and Complicated by Sinusoidal Obstruction Syndrome
  The Israel Medical Association journal : IMAJ · Europe PMC
• 2026-06病例报告
  POEMS syndrome: A case report
  Radiology case reports · DOI · Europe PMC
• 2026-06
  Factors associated with improvement in gait disturbance of polyneuropathy in POEMS syndrome
  BMC neurology · DOI · Europe PMC
• 2026-06
  POEMS syndrome masquerading as allergic cutaneous vasculitis
  The American journal of medicine · DOI · Europe PMC
• 2026-06综述病例报告
  Ultrasound-Guided Sacral Erector Spinae Plane Block for Anorectal Surgery in a Patient with POEMS Syndrome: A Case Study and Literature Review
  A&A practice · DOI · Europe PMC
• 2026-06综述病例报告开放获取
  Successful treatment of idiopathic multicentric Castleman disease with TAFRO and kidney involvement: case report and literature review
  Renal failure · DOI · Europe PMC
• 2026-06病例报告开放获取
  Unicentric Adrenal Castleman Disease Mimicking an Adrenal Tumour
  Cureus · DOI · Europe PMC
• 2026-06综述开放获取
  Monoclonal Antibodies and Derivatives: Therapeutic Tools for Cancer
  Oncology research · DOI · Europe PMC
• 2026-06病例报告
  Optic disc oedema in a patient with POEMS syndrome associated with Castleman's disease
  Archivos de la Sociedad Espanola de Oftalmologia · DOI · Europe PMC
• 2026-06综述病例报告开放获取
  Idiopathic multiple castleman disease case combined with severe neuropathy, Sjogren's syndrome and membrane nephropathy treated by rituximab: a case report and literature review
  Frontiers in immunology · DOI · Europe PMC
• 2026-06
  Fulminant Evans Syndrome and Splenic Infarction As Initial Manifestations of Poems Syndrome with Monoclonal Kappa Light Chain Restriction: A Diagnostic and Therapeutic Challenge
  European journal of case reports in internal medicine · DOI · Europe PMC

在中国开展的临床试验 7L2

按病名在 ClinicalTrials.gov 检索、并校验研究中心含中国大陆而来。登记状态不等于现在真的能入组——务必按 NCT 号到原站核实，并与主治医生商量。

登记为可入组 5

• 招募中NCT04561557
  Safety and Efficacy of CT103A Cells for Relapsed/Refractory Antibody-associated Inflammatory Diseases of the Nervous System
  早期 I 期 · 干预性 · 2020/09/22Tongji Hospital
  中国研究中心 1 个：Wuhan
• 招募中NCT05283993
  A Cohort Study of Plasma Cell Disorders (PCDs) in PKUFH
  观察性 · 2021/07/01Peking University First Hospital
  中国研究中心 1 个：Beijing
• 尚未开始招募NCT06518876
  A Study of KQ-2003 CAR-T Cell Therapy for Patients With Relapsed or Refractory POEMS Syndrome
  I 期 · 干预性 · 2024/08/31Novatim Immune Therapeutics (Zhejiang) Co., Ltd.
  中国研究中心 1 个：Beijing
• 尚未开始招募NCT07115654
  BCMA/CD3 BsAb Therapy for POEMS Syndrome
  II 期 · 干预性 · 2025/08/10Institute of Hematology & Blood Diseases Hospital, China
  中国研究中心 1 个：Tianjin
• 招募中NCT07652879
  KCD (Carfilzomib/Cyclophosphamide/Dexamethasone) Regimen for the Treatment of Newly Diagnosed POEMS Syndrome
  II 期 · 干预性 · 2026/06/15Shanghai Changzheng Hospital
  中国研究中心 1 个：Shanghai

中国境外的在招试验 9L2

CT.gov 报告命中 9 项，此处取回并展示最近的 4 项。

• 招募中NCT07448935
  One Gene, Two Diseases: the Pathologic Role of IGLV1-44 in AL Amyloidosis and POEMS
  观察性 · 2025/11/28Fondazione IRCCS Policlinico San Matteo di Pavia
  意大利
• 招募中NCT06040567
  Polyneuropathy, Impairments and Physical Activity - The PolyImPAct Study
  观察性 · 2023/09/23Rigshospitalet, Denmark
  丹麦
• 招募中NCT06252948
  Gut Microbiome Studies in Patients With POEMS Syndrome and Other Plasma Cell Disorders
  观察性 · 2021/12/29Mayo Clinic
  美国
• 招募中NCT03717844
  Registry for Adults With Plasma Cell Disorders (PCD's)
  观察性 · 2018/02/09UNC Lineberger Comprehensive Cancer Center
  美国

外部标识与链接