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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.

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/c9b129aa2373/cureus-0016-00000075620-i01.jpg

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