APL的发病分子机制和治疗进展

Molecular Pathogenesis and Current Therapeutic Approaches of APL

急性前髓细胞性白血病(APL)是急性髓细胞样白血病(AML)的一个亚型,它的分子生物学特征为95%的患者有15号染色体前髓细胞性白血病基因(PML)与17号染色体视黄酸受体(RARα)基因的融合易位表达。维甲酸(ATRA)单药治疗能使APL患者达90%的缓解率,化疗药物与ATRA的联合应用更能降低患者复发率,改善生存;三氧化二砷(ATO)能使复发患者缓解率达到90%。这篇文章主要综述APL的发病分子机制和治疗进展。

Acute promyelocytic leukemia （APL） is a subtype of acute myeloid leukemia （AML） with a distinct biology and clinical presentation. The vast majority of cases are characterized by a translocation that fuses the promyelocytic leukemia gene （PML） on chromosome 15 with a gene encoding a retinoic acid receptor （RARc0 on chromosome 17. As a single agent, all-trans retinoic acid （A- TRA） has produced complete remissions in up to 90% of patients, and the use of ATRA in combination with chemotherapy has significantly reduced relapse rates and improved survival. Similarly, arsenic trioxide （ATO） has produced remission in up to 90% of patients with relapsed APL. This article examined the molecular pathogenesis as it relates to the diagnosis and monitoring of APL and review current therapeutic approaches.