儿童朗格汉斯细胞组织细胞增生症发病机制研究进展  

Progress on the pathogenesis of Langerhans cell histiocytosis in children

作　　者：泥永安[1] Ni Yongan(Department of Pediatric Hematology and Oncology,The Affiliated Hospital of Qingdao University,Qingdao 266555,China)

机构地区：[1]青岛大学附属医院儿童血液肿瘤科,266555

出　　处：《国际儿科学杂志》2025年第3期189-193,共5页International Journal of Pediatrics

摘　　要：朗格汉斯细胞组织细胞增生症(Langerhans cell histiocytosis,LCH)是来源于髓系的炎性恶性肿瘤,发病机制尚未完全阐明。研究显示LCH发病机制中涉及RAS-RAF-MEK-ERK信号通路、PI3K-AKT信号通路和NOTCH信号通路,主要是RAS-RAF-MEK-ERK信号通路,PI3K-AKT信号通路的激活与RAS-RAF-MEK-ERK信号通路的激活有关,但ERK激活后导致LCH形成的具体机制不清,且朗格汉斯细胞的起源也存在争议。另外,肿瘤微环境在LCH的发病机制中也发挥重要作用,同时免疫检查点程序性细胞死亡1配体-1、白细胞介素-17A、融合基因PLEKH26-NTRK3及粒细胞集落刺激因子1受体可能也参与LCH的发病。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.

关 键 词：朗格汉斯细胞组织细胞增生症 发病机制 BRAF V600E 程序性细胞死亡1配体-1 肿瘤微环境 

分 类 号：R725.9[医药卫生—儿科]