[ Erdheim-Chester病的诊断与治疗——综述]

[Diagnosis and Treatment of Erdheim-Chester Disease -Review].

作者信息

Wang Jing-Shi, Wang Zhao

机构信息

Department of Hematology, Beijing Friendship Hospital Affiliated to Capital Medical University, Beijing 100050, China.

Department of Hematology, Beijing Friendship Hospital Affiliated to Capital Medical University, Beijing 100050, China.E-mail:

出版信息

Zhongguo Shi Yan Xue Ye Xue Za Zhi. 2016 Aug;24(4):1256-9. doi: 10.7534/j.issn.1009-2137.2016.04.055.

DOI:10.7534/j.issn.1009-2137.2016.04.055

PMID:27531811

Abstract

Erdheim-Chester Disease (ECD) is a rare form of non Langerhans' cell histiocytosis. The etiology of ECD is unknown yet. The disease is characterized by the infiltration of lipid-laden histiocytes with foamy to bones and various organs. The heterogeneous manifestations of ECD vary among different individuals. This results in a presentation that may vary from an indolent focal disease to a life threatening organ failure. This review focuses on the etiology, clinical presentation, diagnostic criteria and treatment of Erdheim-Chester disease (ECD).

摘要

厄德里希-切斯特病（ECD）是一种罕见的非朗格汉斯细胞组织细胞增多症。ECD的病因尚不清楚。该疾病的特征是富含脂质的组织细胞浸润，这些细胞呈泡沫状，可累及骨骼和各种器官。ECD的临床表现具有异质性，在不同个体之间有所不同。这导致其表现可能从惰性局灶性疾病到危及生命的器官衰竭不等。本综述重点关注厄德里希-切斯特病（ECD）的病因、临床表现、诊断标准和治疗。