摘要Ph样急性淋巴细胞白血病(ALL)是一组前体B细胞急性淋巴细胞白血病(BCP-ALL)亚型,好发于儿童及成年人,并且一般预后差.该亚型与断裂点簇集区-Abelson白血病病毒(BCR-ABL) 1+ ALL有相似的基因表达谱,但是缺乏BCR-ABL1融合蛋白,基因异常主要涉及细胞因子信号通路、激酶信号通路,以及B细胞发育相关转录因子.激酶抑制剂联合化疗的治疗方案有望改善Ph样ALL的预后.通过对Ph样ALL的研究,有助于完善BCP-ALL的精确危险分层和靶向治疗.本文就Ph样ALL的流行病学特点、易感基因位点、常见基因异常、诊断、治疗及预后的研究进展进行综述.

abstractsPh-like acute lymphoblastic leukemia (ALL) is a subtype of B cell precursor acute lymphoblastic leukemia (BCP ALL) with poor outcome that exhibits a gene expression profile similar to breakpoint cluster region-Abelson leukemia virus (BCR-ABL)1-positive ALL but lacks the BCR-ABL1 fusion protein.The gene abnormalities involve of cytokine signaling pathway,kinase signaling pathway,and B cell developmental transcription factors.Protein kinase inhibitors combined with chemotherapy might be applied in Ph-like ALL.The investigation of Ph-like ALL will contribute to precise risk stratification of BCP-ALL and targeted therapy.The research progress of Ph-like ALL will be introduced in this article,including epidemiology,susceptibility locus,gene variants,diagnosis,treatment and prognosis.