POEMS（多发性神经病、脏器肿大、内分泌病、M蛋白、皮肤病变）综合征：一份来自南美洲的报告。

POEMS (polyneuropathy, organomegaly, endocrinopathy, M protein, skin lesions) syndrome: a South America's report.

作者信息

Leite Ana Claudia Celestino, Nascimento Osvaldo J M, Lima Marco Antonio, Andrada-Serpa Maria José

机构信息

Postgraduate Program on Neurology / Neuroscience from The Fluminense Federal University.

出版信息

Arq Neuropsiquiatr. 2007 Jun;65(2B):516-20. doi: 10.1590/s0004-282x2007000300030.

DOI:10.1590/s0004-282x2007000300030

PMID:17665027

Abstract

The POEMS syndrome, also known as Crow-Fukase syndrome, is an unusual systemic disorder described mainly in Asian individuals. It is characterized by the presence of (P)polyneuropathy, (O)organomegaly, (E)endocrinopathy, (M) M-protein, and (S) skin changes. Several other associated conditions such as sclerotic bone lesions, Castleman disease, low-grade fever, edema and hematologic disorders are usually seen. We describe five Brazilian patients with this syndrome. Two patients presented Castleman disease, one patient presented osteosclerotic myeloma and in two patients no associated conditions were found.

摘要

POEMS综合征，也称为克劳-深泽综合征，是一种主要在亚洲人群中描述的罕见全身性疾病。它的特征是存在（P）多发性神经病、（O）脏器肿大、（E）内分泌病、（M）M蛋白和（S）皮肤改变。通常还可见其他几种相关病症，如硬化性骨病变、卡斯尔曼病、低热、水肿和血液系统疾病。我们描述了五例患有该综合征的巴西患者。两名患者患有卡斯尔曼病，一名患者患有骨硬化性骨髓瘤，两名患者未发现相关病症。