Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, 100730, China.
State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.
Cell Commun Signal. 2022 Dec 19;20(1):195. doi: 10.1186/s12964-022-00917-0.
Langerhans cell histiocytosis (LCH) is an inflammatory myeloid malignancy in the "L-group" histiocytosis. Mitogen-activated protein kinase (MAPK) pathway activating mutations are detectable in nearly all LCH lesions. However, the pathogenic roles of MAPK pathway activation in the development of histiocytosis are still elusive. This review will summarize research concerning the landscape and pathogenic roles of MAPK pathway mutations and related treatment opportunities in Langerhans cell histiocytosis. Video abstract.
朗格汉斯细胞组织细胞增生症(LCH)是“L 组”组织细胞增生症中的一种炎症性髓系恶性肿瘤。几乎所有 LCH 病变中都可检测到丝裂原活化蛋白激酶(MAPK)通路激活突变。然而,MAPK 通路激活在组织细胞增生症发展中的致病作用仍不清楚。这篇综述将总结关于 MAPK 通路突变的景观和致病作用以及 Langerhans 细胞组织细胞增生症相关治疗机会的研究。视频摘要。