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.
Keywords: Inflammatory myeloid malignancy; Langerhans cell histiocytosis (LCH); MAPK pathway; MEK inhibitors; Progenitor cells; RAF inhibitors.
© 2022. The Author(s).