Abstract
Langerhans-cell histiocytosis (LCH), the most common histiocytic disorder, is a reactive clonal proliferation and accumulation of CD1a+/CD207+ dendritic cells in inflammatory lesion, characterized by its strong heterogeneity and changeable complexity including apparent inflammation and tumor features,which should be redefined as an inflammatory myeloid neoplasia. Recent research has shown that LCH is the consequence of misguided myeloid differentiation on account of genomic aberrances in the RAS-RAF-MEK-ERK pathway. These gene inhibitors may present more curative effects for the treatment of LCH. With further prospective clinical trial, molecular targeted therapy may combine with or even replace the traditional surgery plus chemotherapy as the first-line regimen in LCH. Key words: Histiocytosis, Langerhans-cell; Dendritic cells; Gene, BRAF; Molecular targeted therapy
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