Department of Pediatrics, West China Second University Hospital, Sichuan University, Chengdu, China.
Int J Hematol. 2010 May;91(4):708-10. doi: 10.1007/s12185-010-0553-5. Epub 2010 Apr 20.
The t(5;17)/NPM-RARalpha is the second variant chromosomal translocation in acute promyelocytic leukemia (APL) to be characterized and also the second most plentiful variant translocation. So far, there is a lack of information on the effectiveness of arsenic trioxide (ATO) in relapsed APL with variant RARalpha chimera including t(5;17)/NPM-RARalpha. We report here a long-term survived APL patient with variant NPM-RARalpha fusion who relapsed four times and each time responded well to ATO or ATO-based re-induction therapy. The patient had received a total of more than 3,500 mg of ATO, but showed no obvious arsenic-related toxicities. This case illustrates the long-term efficiency and safety of ATO-based therapy not only in newly diagnosed APL, but also in relapsed APL including those with variant translocations.
t(5;17)/NPM-RARα 是继 t(15;17)/PML-RARα 之后被鉴定的第二个急性早幼粒细胞白血病（APL）变异染色体易位，也是第二个最常见的变异易位。到目前为止，关于三氧化二砷（ATO）在包括 t(5;17)/NPM-RARα 在内的变异型 RARα 嵌合体复发性 APL 中的疗效尚缺乏信息。我们在此报告一例长期生存的变异型 NPM-RARα 融合的 APL 患者，该患者复发了 4 次，每次均对 ATO 或基于 ATO 的再诱导治疗反应良好。该患者共接受了超过 3500mg 的 ATO，但未出现明显的砷相关毒性。该病例说明基于 ATO 的治疗不仅在新诊断的 APL 中，而且在包括变异型易位在内的复发性 APL 中均具有长期的疗效和安全性。
