朗格汉斯细胞组织细胞增多症的研究进展

Progress in Langerhans-cell histiocytosis

朗格汉斯细胞组织细胞增多症（LCH）是最常见的组织细胞增生症，是以 CD1a＋／CD207＋树突状细胞异常克隆增生的一类疾病。 LCH 临床表现复杂多变，异质性强，既有明显的炎症反应又有肿瘤的特性，其应被重新定义为炎症性髓系肿瘤。最近的研究发现 LCH 是髓系树突状细胞前体误分化的结果，而误分化的原因是 RAS-RAF-MEK-ERK 通路中的基因突变。采用这些基因抑制剂治疗 LCH 可能会有较好效果。随着前瞻性临床试验的进一步开展，分子靶向治疗可能会联合甚至取代传统的手术、化疗，成为 LCH 临床一线治疗方案。

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.