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[伴有多中心Castleman病的不完全性POEMS综合征]

[Incomplete POEMS syndrome with multicentric Castleman's disease].

作者信息

González de la Aleja P, García-Navarro M, Sánchez-Martínez R, Ramos J M

机构信息

.

出版信息

An Sist Sanit Navar. 2017 Dec 29;40(3):475-478. doi: 10.23938/ASSN.0041.

Abstract

Castleman's disease (CD) is an atypical lymphoproliferative disorder of unknown cause, characterized by non-clonal nodal hyperplastic growth. Two forms of clinical presentation are currently recognized, one localized and the other multicentric, and four histopathologic variants. It is characterized by generalized lymphadenopathy, hepatosplenomegaly, fever and night sweats. CD may present severe pancytopenia, multi-organ failure, lymphoma evolution and it can sometimes be associated with paraneoplastic syndromes such as POEMS syndrome. Associations of these two entities have been widely described in the current literature, although its less common association with amyloidosis is described as isolated clinical cases. We report a case with this triple association: EC, POEMS and amyloidosis.

摘要

卡斯尔曼病(CD)是一种病因不明的非典型淋巴增生性疾病,其特征为非克隆性淋巴结增生性生长。目前已认识到两种临床表现形式,一种为局限性,另一种为多中心性,以及四种组织病理学变异型。其特征为全身性淋巴结肿大、肝脾肿大、发热和盗汗。CD可能出现严重全血细胞减少、多器官功能衰竭、淋巴瘤演变,有时还可与副肿瘤综合征如POEMS综合征相关联。这两种疾病的关联在当前文献中已有广泛描述,尽管其与淀粉样变性较少见的关联仅作为个别临床病例被报道。我们报告一例存在这种三联关联的病例:内分泌病(EC)、POEMS综合征和淀粉样变性。

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