脂质肉芽肿病诊断及治疗进展

Diagnosis and Treatment of Erdheim-Chester Disease——Review

肉芽肿病(Erdheim-Chester Disease,ECD)是一种罕见的非朗格汉斯细胞组织细胞增生症,其病因和发病机制仍然不清,以富含脂质的泡沫细胞侵犯骨骼及多种器官为特征。无特异性临床表现,可从局部病变到危及生命的器官衰竭。本文对ECD的发病机制、临床表现、诊断标准和治疗作一综述。

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).