Erdheim-Chester病一例报告及文献回顾  

Erdheim-Chester disease(ECD):a case report and literature review

作　　者：舒玉炳[1] 施鑫[1] 冯思坛[1] 周光新[1] 

机构地区：[1]南方医科大学南京临床学院(南京军区南京总医院)骨科,210002

出　　处：《中国骨与关节杂志》2012年第5期551-553,共3页Chinese Journal of Bone and Joint

摘　　要：Erdheim-Chester疖i（Erdheim—Chesterdisease，ECD）是一种病因不明罕见的非郎格罕组织细胞增生症，以泡沫样细胞侵犯组织形成脂质肉芽肿为主要特点。常侵犯骨骼，也可累及骨骼外组织。现今世界范围内约有440多例报告。因ECD发病率低，临床表现形式多样，诊治困难，易被误诊。为提高对该疾病的了解和认识，现就我院1例ECD进行报告和相关文献进行回顾。Erdheim-Chester disease （ECD） is a rare, non-Langerhans form of histiocytosis characterized by xanthomatous tissue infiltration with foamy histocytes. The etiology is unknown. It has a wide spectrum of clinical manifestations and the long bone is the primary site of involvement with the characteristic of bilateral, diffuse cortical sclerosis. The lesion in the femur and tibia is more common, the bone pain and arthralgias are the most frequent symptoms. Different forms of extraskeletal sites may be frequently involved. The diagnose and treatment of ECD are difficult and the prognosis is bad with a progressive deterioration and easy misdiagnose. In order to improve the understanding of the disease, a case of ECD in our hospital is reported and literatures are reviewed.

关 键 词：文献回顾 郎格罕组织细胞增生症 病因不明 组织形成 世界范围 临床表现 ECD 肉芽肿 

分 类 号：R681[医药卫生—骨科学]