骨骼朗格汉斯细胞增生症的治疗进展  

Literature review of the treatment of Langerhans cell histiocytosis

作　　者：杜鑫辉[1] 董扬[1] 杨庆诚[1] 

机构地区：[1]上海交通大学附属第六人民医院骨科,200233

出　　处：《中国骨与关节杂志》2012年第3期293-295,共3页Chinese Journal of Bone and Joint

摘　　要：朗格汉斯细胞增生症是以朗格汉斯细胞异常累积为特征的疾病,其发病原因尚不明确,发病率约为1∶1 500 000.患者以儿童为主,男性多见.根据朗格汉斯细胞增生症病灶累及部位及临床表现不同可分为嗜酸性肉芽肿,汗雪氏病（Hand-Schüller-Christian disease）及里斯氏病（Letterer-Siwe disease）.Langerhans cell histiocytosis （LCH） is a poorly understood disease, with different patterns of clinical presentation. There are many methods concerning the treatment of Langerhans cell histiocytosis, but the optimal choice is still to be argued. Here we present a literature review of all the papers included by WEB of KNOWLEDGE of the late 10 years on the clinical features, diagnostic methods and especially, the treatment of Langerhans cell histiocytosis. Based on the location of the lesion and clinical features, LCH can be classified into eosinophilic granuloma, Hand-Schuller-Christian disease and Letterer-Siwe disease. The symptoms vary according to the affected lesion, which can be asymptomatic or pain. LHC can affect any bone of the body, usually appearing as a radiolucent area with a sclerosis in the diaphysis or metaphysis of long bone. LHC of the spine often presents with vertebra body destruction and compression. The radiographic studies could provide evidence that indicating the diagnosis of LHC, the final decision is usually made by pathologist by studying the biopsy specimen. The treatment of LHC includes chemotherapy, radiotherapy, intralesional injection of steroids and surgery. Each method has its own indications and the treatment of patients should be individualized.

关 键 词：朗格汉斯细胞增生症 治疗 骨骼 嗜酸性肉芽肿 发病原因 临床表现 发病率 

分 类 号：R55[医药卫生—血液循环系统疾病]