Progress in the Treatment of Adult Langerhans Cell Histiocytosis --Review.
10.19746/j.cnki.issn.1009-2137.2022.03.050
- Author:
Zi-Zhu TIAN
1
;
Ya-Juan CUI
1
;
Hong-Ling PENG
2
Author Information
1. Department of Hematology， The Second Xiangya Hospital of Central South University, Changsha 410011, Human Province, China.
2. Department of Hematology， The Second Xiangya Hospital of Central South University, Changsha 410011, Human Province, China,E-mail：penghongling@csu.edu.cn.
- Publication Type:Review
- Keywords:
Langerhans cell histiocytosis;
adult;
immunological;
targeted;
treatment
- MeSH:
Adult;
Child;
Histiocytosis, Langerhans-Cell/therapy*;
Humans;
Mutation;
Proto-Oncogene Proteins B-raf/metabolism*;
Signal Transduction;
T-Lymphocytes, Regulatory/pathology*
- From:
Journal of Experimental Hematology
2022;30(3):970-974
- CountryChina
- Language:Chinese
-
Abstract:
Langerhans cell histiocytosis (LCH) is a rare proliferative disease dominated by the proliferation of Langerhans cells, which is inflammatory myeloid neoplasms. Its clinical manifestations are variable, occurring at any age and at any site, and it is rarer in adults than in children. The gold standard for diagnosis is histopathological biopsy. Due to the rarity of adult LCH and the heterogeneity of this disease, treatment of adult LCH should be developed according to the extent of the disease and risk stratification. With the discovery of MAPK, PI3K and c-KIT signaling pathway activation, especially BRAF V600E and MAP2K1 mutations, targeted therapy has become a hot spot for therapeutic research. Meanwhile, the discovery of high expression of M2-polarized macrophages and Foxp3+ regulatory T cells (Treg) in LCH has provided an important basis for the immunotherapy. In this article, we will focus on reviewing the latest research progress in the treatment of adult LCH in recent years, and provide a reference for clinical research on the treatment of adult LCH patients.
