Abstract
朗格汉斯细胞组织细胞增生症（LCH）是一种罕见的组织细胞肿瘤，儿童发病率为（3~5）/100万，成人发病率不足1/100万。LCH致死率低，但可引起严重的器官功能障碍。目前尚无公认针对成人患者的治疗推荐。自2013年欧洲组织细胞协会发布了成人LCH的管理建议以来，近年陆续报道了多项成人LCH队列研究。此外，随着丝裂原活化蛋白激酶（MAPK）途径中的基因突变陆续被发现，各种新型靶向药物也逐渐投入使用。本文就近10年成人LCH的治疗进展进行综述。.
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