朗格汉斯细胞组织细胞增多症
发病机制
组织细胞增多症
疾病
突变
生物
癌症研究
MAPK/ERK通路
医学
激酶
免疫学
病理
基因
细胞生物学
遗传学
作者
Tommaso Sconocchia,Johannes Foßelteder,Giuseppe Sconocchia,Andreas Reinisch
标识
DOI:10.3389/fimmu.2023.1275085
摘要
Langerhans cell histiocytosis (LCH) is a rare and clinically heterogeneous hematological disease characterized by the accumulation of mononuclear phagocytes in various tissues and organs. LCH is often characterized by activating mutations of the mitogen-activated protein kinase (MAPK) pathway with BRAFV600E being the most recurrent mutation. Although this discovery has greatly helped in understanding the disease and in developing better investigational tools, the process of malignant transformation and the cell of origin are still not fully understood. In this review, we focus on the newest updates regarding the molecular pathogenesis of LCH and novel suggested pathways with treatment potential.
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