朗格汉斯细胞组织细胞增生症（LCH）是一种以大量未成熟树突状细胞在组织中异常积累为特征的罕见疾病。 LCH的病因与发病机制随着BRAFV600E基因突变的发现已逐渐明朗，但LCH多样化的临床表现仍导致其治疗困难。文 章综述近年来有关LCH的病因与发病机制和治疗的最新进展。

　Langerhans cell histiocytosis (LCH) is a rare disease characterized by abnormal accumulation of immature dendritic cells in tissues. With the discovery of BRAFV600E gene mutation, the etiology and pathogenesis of LCH have become clear, but the diverse clinical manifestations of LCH still result in difficulties of its treatment. This article reviews the latest progress in the etiology, pathogenesis and treatment of LCH in recent years.