成人朗格汉斯细胞组织细胞增生症累及肺、垂体、甲状腺、肝、淋巴结、皮肤1例  

Adult Langerhans cell histiocytosis involving lung,pituitary, thyroid, liver, lymph nodes, and skin:A case report

作　　者：刘贵钱 刘婷 沈冲 周莉[1] 欧阳若芸[1] LIU Guiqian;LIU Ting;SHEN Chong;ZHOU Li;OUYANG Ruoyun(Department of Respiratory and Critical Care Medicine,Second Xiangya Hospital,Central South University,Changsha 410011,China)

机构地区：[1]中南大学湘雅二医院呼吸与危重症医学科

出　　处：《中南大学学报（医学版）》2020年第1期96-101,共6页Journal of Central South University ：Medical Science

基　　金：国家重点临床专科建设项目(2012-650);国家重点研发计划(2016YFC0901502);湖南省财政厅产业研发项目(2015-83)~~

摘　　要：朗格汉斯细胞组织细胞增生症是一种少见且病因未明的疾病,可以累及全身任何器官或系统,临床表现多样。1例31岁男性患者,有吸烟史,以干咳、多饮、多尿起病,期间多次出现自发性气胸,肺高分辨CT表现为弥漫性囊性及小结节病变,经冷冻肺活检诊断为朗格汉斯细胞组织细胞增生症。查找其他系统受累情况,发现肺、垂体、甲状腺、肝、淋巴结、皮肤受累。治疗多采用糖皮质激素或全身化学药物治疗,BRAF基因突变抑制剂是新的治疗方向。Langerhans cell histiocytosis(LCH)is a rare and unexplained disease that can involve in any organ or system in the body and displays a variety of clinical manifestations.A 31-yearold man,who had a more than 10-year smoke history,initially presented dry cough,polydipsia and diuresis,with recurrent spontaneous pneumothorax.Pulmonary highresolution computed tomography showed diffuse cystic and nodular lesions.Langerhans cell histiocytosis was confirmed by a transbronchial cryobiopsy.The disease is involved in the lung,pituitary,thyroid,liver,lymph node,and skin.Glucocorticoid or systemic chemotherapy is commonly used in the treatment for this disease.BRAF gene mutation inhibitor is a new direction for the treatment.

关 键 词：朗格汉斯细胞组织细胞增生症 多系统 诊断 治疗 

分 类 号：R597[医药卫生—内科学]