儿童朗格汉斯细胞组织细胞增生症病因和治疗进展

doi:10.3969/j.issn.1000-3606.2019.03.017

临床儿科杂志 ›› 2019, Vol. 37 ›› Issue (3): 228-.doi: 10.3969/j.issn.1000-3606.2019.03.017

儿童朗格汉斯细胞组织细胞增生症病因和治疗进展

方凯弘,　徐倩玥综述,　余红审校

上海交通大学医学院附属新华医院皮肤科（上海　200092）

出版日期:2019-03-15 发布日期:2019-03-25

Advances in etiology and treatment of Langerhans cell histiocytosis in children

Reviewer: FANG Kaihong, XU Qianyue, Reviser: YU Hong

Department of Dermatology, Xinhua Hospital Affiliated to Shanghai Jiao Tong University School of Medicine, Shanghai 200092, China

Online:2019-03-15 Published:2019-03-25

摘要/Abstract

摘要： 　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。

关键词: 朗格汉斯细胞组织细胞增生症；　病因；　BRAFV600E基因突变；　治疗

Abstract: 　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.

Key words: 　Langerhans cell histiocytosis;　etiology;　BRAFV600E mutation;　treatment

方凯弘，徐倩玥. 儿童朗格汉斯细胞组织细胞增生症病因和治疗进展[J]. 临床儿科杂志, 2019, 37(3): 228-.

FANG Kaihong, XU Qianyue. Advances in etiology and treatment of Langerhans cell histiocytosis in children[J]. Journal of Clinical Pediatrics, 2019, 37(3): 228-.

儿童朗格汉斯细胞组织细胞增生症病因和治疗进展

Advances in etiology and treatment of Langerhans cell histiocytosis in children

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