Treatment Progress of Langerhans'Cell Histiocytosis in Children
10.3870/j.issn.1004-0781.2025.12.015
- VernacularTitle:儿童朗格汉斯细胞组织细胞增生症的治疗进展
- Author:
Yu CHEN
1
;
Aiguo LIU
1
Author Information
1. 华中科技大学同济医学院附属同济医院儿童血液科,武汉 430030
- Publication Type:Journal Article
- Keywords:
Langerhans'cell histiocytosis in children;
Chemotherapy;
Targeted therapy
- From:
Herald of Medicine
2025;44(12):1975-1981
- CountryChina
- Language:Chinese
-
Abstract:
Langerhans cell histiocytosis(LCH)is an inflammatory myeloid neoplastic disease characterized by abnormal proliferation of pathologic Langerhans cells that are CD1a+/CD207+,and its onset is closely associated with abnormal activation of the MAPK pathway.According to international stratification criteria,LCH is classified into single-system LCH(SS-LCH)and multi-system LCH(MS-LCH),with the latter being associated with poor prognosis when it involves the liver,spleen,or hematopoietic system.The treatment strategy for LCH in children is based on risk stratification,and currently,vinblastine combined with prednisone is the standard first-line treatment;a unified standard for second-line treatment has not been established.Cytarabine,cladribine,and clofarabine are the main treatment drugs.Targeted drugs BRAF and MEK inhibitors have shown high efficacy in clinical treatment,and clinical studies on their administration methods and combination therapies are currently hot topics.Immunotherapy and hematopoietic stem cell transplantation(HSCT)have shown potential in relapsed and refractory cases,warranting further exploration.The article systematically reviews the progress in the treatment of pediatric LCH,providing reference for clinical and research purposes.
