[Chinese expert consensus on the diagnosis and treatment of adult Langerhans cell histiocytosis (2025)].

Abstract

Langerhans cell histiocytosis (LCH) is a clonal hematologic neoplasm primarily characterized by the activation of the mitogen-activated protein kinase (MAPK) signaling pathway; it can involve any organ or system and presents with diverse clinical manifestations. Based on current evidence-based medicine and combined with domestic clinical experience, this consensus elaborates on the pathogenesis, clinical manifestations, diagnosis, treatment, and efficacy evaluation of adult LCH, aiming to provide professional guidance for its diagnosis and treatment in China.

朗格汉斯细胞组织细胞增生症（LCH）是一种以丝裂原活化蛋白激酶（MAPK）信号通路激活为主要分子特征的克隆性血液系统肿瘤，可累及全身各器官及系统，临床表现多样。基于现有循证医学证据，结合国内临床实践经验，本共识从成人朗格汉斯细胞组织细胞增生症的发病机制、临床表现、诊断、治疗、疗效评估等内容进行阐述，以期为中国成人朗格汉斯细胞组织细胞增生症诊断和治疗提供专业指导意见。.