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一名10个月大男孩表现为全血细胞减少的X连锁淋巴增生性疾病。

X-linked lymphoproliferative disease presenting as pancytopenia in a 10-month-old boy.

作者信息

Chadha S Nicole, Amrol David

机构信息

Departments of Pediatrics and Internal Medicine, University of South Carolina School of Medicine, 14 Medical Park Suite 400, Columbia, SC 29203, USA.

出版信息

Case Rep Med. 2010;2010:517178. doi: 10.1155/2010/517178. Epub 2010 Jun 21.

Abstract

X-linked lymphoproliferative disease, also known as Duncan's syndrome, is a rare genetic disorder that causes exaggerated immune responses to Epstein-Barr virus (EBV) infection and often leads to death. Patient presentation varies but can include signs and symptoms typical of EBV, pancytopenia, and fulminant hepatitis.

摘要

X连锁淋巴增生性疾病,也称为邓肯综合征,是一种罕见的遗传性疾病,可导致对爱泼斯坦-巴尔病毒(EBV)感染产生过度免疫反应,并常导致死亡。患者的表现各不相同,但可能包括EBV感染的典型体征和症状、全血细胞减少症和暴发性肝炎。

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