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解析多神经病、器官肿大、内分泌病、单克隆丙种球蛋白病和皮肤改变综合征:国家三级医疗中心的诊断挑战与治疗策略

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

作者信息

Zambrano Zambrano Kevin, Martinez Salazar Julio, Velarde Michaud Jason Cedric, Montalvo Lopez Gavito Arantxa, Zambrano Zambrano Alexis

机构信息

Internal Medicine, National Medical Center November 20, Mexico City, MEX.

Hematology, National Medical Center November 20, Mexico City, MEX.

出版信息

Cureus. 2024 Dec 12;16(12):e75620. doi: 10.7759/cureus.75620. eCollection 2024 Dec.

DOI:10.7759/cureus.75620
PMID:39803068
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11725331/
Abstract

Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal Gammopathy, and Skin Changes (POEMS) syndrome is a rare paraneoplastic disorder caused by plasma cell proliferation and overproduction of cytokines, particularly vascular endothelial growth factor (VEGF). This complex syndrome affects multiple organ systems and presents with a broad range of clinical and laboratory manifestations, which can complicate both diagnosis and management. Not all components of the acronym are observed in every patient, highlighting the clinical heterogeneity of the condition. The case discussed herein illustrates the diagnostic approach to a patient suspected of having POEMS syndrome.

摘要

多发性神经病变、器官肿大、内分泌病变、单克隆丙种球蛋白病和皮肤改变(POEMS)综合征是一种罕见的副肿瘤性疾病,由浆细胞增殖和细胞因子过度产生引起,尤其是血管内皮生长因子(VEGF)。这种复杂的综合征会影响多个器官系统,并呈现出广泛的临床和实验室表现,这会使诊断和管理都变得复杂。并非每个患者都会出现该首字母缩写词所包含的所有症状,这突出了该病症的临床异质性。本文讨论的病例说明了对疑似患有POEMS综合征患者的诊断方法。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/271a/11725331/6ec7356a0134/cureus-0016-00000075620-i03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/271a/11725331/c9b129aa2373/cureus-0016-00000075620-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/271a/11725331/bbba1e895591/cureus-0016-00000075620-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/271a/11725331/6ec7356a0134/cureus-0016-00000075620-i03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/271a/11725331/c9b129aa2373/cureus-0016-00000075620-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/271a/11725331/bbba1e895591/cureus-0016-00000075620-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/271a/11725331/6ec7356a0134/cureus-0016-00000075620-i03.jpg

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本文引用的文献

1
POEMS syndrome: Update on diagnosis, risk-stratification, and management.POEMS综合征:诊断、风险分层及管理的最新进展
Am J Hematol. 2023 Dec;98(12):1934-1950. doi: 10.1002/ajh.27081. Epub 2023 Sep 21.
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Bortezomib plus dexamethasone as first-line therapy for patients with POEMS syndrome.硼替佐米联合地塞米松作为 POEMS 综合征患者的一线治疗。
Ann Hematol. 2021 Nov;100(11):2755-2761. doi: 10.1007/s00277-021-04609-6. Epub 2021 Jul 31.
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Update on the Diagnosis and Treatment of POEMS (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal Gammopathy, and Skin Changes) Syndrome: A Review.
POEMS(多发性神经病、器官肿大、内分泌病、单克隆丙种球蛋白病和皮肤改变)综合征的诊断和治疗进展:综述。
JAMA Oncol. 2021 Sep 1;7(9):1383-1391. doi: 10.1001/jamaoncol.2021.0586.
4
Multiple myeloma: EHA-ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up.多发性骨髓瘤:欧洲血液学协会-欧洲肿瘤内科学会诊断、治疗及随访临床实践指南
Ann Oncol. 2021 Mar;32(3):309-322. doi: 10.1016/j.annonc.2020.11.014. Epub 2021 Feb 3.
5
Endocrine Evaluation in POEMS Syndrome: A Cohort Study.POEMS 综合征的内分泌评估:一项队列研究。
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POEMS syndrome: A multisystem clonal disorder.POEMS 综合征:一种多系统克隆性疾病。
Eur J Haematol. 2021 Jan;106(1):14-18. doi: 10.1111/ejh.13514. Epub 2020 Oct 8.
7
Proposal of new clinical diagnostic criteria for POEMS syndrome.POEMS 综合征新的临床诊断标准的建议。
J Neurol Neurosurg Psychiatry. 2019 Feb;90(2):133-137. doi: 10.1136/jnnp-2018-318514. Epub 2018 Oct 2.
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POEMS syndrome.POEMS 综合征。
Curr Opin Neurol. 2018 Oct;31(5):551-558. doi: 10.1097/WCO.0000000000000610.
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POEMS Syndrome: an Enigma.POEMS综合征:一个谜团。
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Prognostic study for overall survival in patients with newly diagnosed POEMS syndrome.新诊断 POEMS 综合征患者总生存预后研究。
Leukemia. 2017 Jan;31(1):100-106. doi: 10.1038/leu.2016.168. Epub 2016 Jun 13.