Progress on the pathogenesis of Langerhans cell histiocytosis in children
10.3760/cma.j.issn.1673-4408.2025.03.010
- VernacularTitle:儿童朗格汉斯细胞组织细胞增生症发病机制研究进展
- Author:
Yongan NI
1
Author Information
1. 青岛大学附属医院儿童血液肿瘤科 266555
- Keywords:
Langerhans cell histiocytosis;
Pathogenesis;
BRAF V600E;
Programmed cell death-1 ligand 1;
Tumor microenvironment
- From:
International Journal of Pediatrics
2025;52(3):189-193
- CountryChina
- Language:Chinese
-
Abstract:
Langerhans cell histiocytosis（LCH）is an inflammatory malignant tumor originating from the myeloid system，and its pathogenesis has not been fully elucidated.Research has shown that the pathogenesis of LCH involves the RAS-RAF-MEK-ERK signaling pathway，PI3K-AKT signaling pathway，and NOTCH signaling pathway，mainly the RAS-RAF-MEK-ERK signaling pathway.The activation of the PI3K-AKT signaling pathway is related to the activation of the RAS-RAF-MEK-ERK signaling pathway，but the specific mechanism by which ERK activation leads to LCH formation is unclear，and the origin of Langerhans cells is also controversial.Additionally，the tumor microenvironment plays an important role in the pathogenesis of LCH，and immune checkpoint programmed cell death-1 ligand 1，interleukin-17A，fusion gene PLEKH26-NTRK3，and granulocyte colony-stimulating factor 1 receptor may also be involved in the pathogenesis of LCH.
