急性早幼粒细胞白血病发病机制研究新进展

Research Progress in Pathogenesis of Acute Promyelocytic Leukemia

急性早幼粒细胞白血病的发病机制已有较深入研究,经典的发病机制是由于染色体t(15;17)易位形成PML/维甲酸受体α(RARα)融合基因,进而产生PML/RARα融合蛋白导致疾病的发生。近年来随着研究的进展,更多的融合基因被发现,且有相对各异的性质。虽然经典的PML/RARα占急性早幼粒细胞白血病的大多数,但其他类型基因对药物敏感性却各有不同,应明确其发病机制,指导治疗。

There has been deep research about the pathogenesis of acute promyelocytic leukemia. The classical pathogenesis is due to the translocation of chromosome t（ 15; 17）,the formation of promyelocyticleukemia（ PML） / retinoic acid receptor α（ RARα） fusion gene and then the generation of PML / RARαfusion protein which leads to the occurrence of the disease. In recent years,with the development of the research,more fusion genes are discovered,with relatively different natures. Although the classic PML / RARαaccounts for the majority of acute promyelocytic leukemia,other types have lots of differences in drug sensitivity,so the pathogenesis should be made clear so as to instruct the treatment.