The clinical spectrum of adult acute myeloid leukaemia associated with core binding factor translocations
The clinical spectrum of adult acute myeloid leukaemia associated with core binding factor translocations
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DOI：
10.1111/j.1365-2141.2006.06276.x
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发表时间：
2006-10-01
影响因子：
6.5
通讯作者：
Estey, Elihu H.
中科院分区：
文献类型：
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作者：
Appelbaum, Frederick R.;Kopecky, Kenneth J.;Estey, Elihu H.
为了更好地了解与核心结合因子（CBF）易位相关的成人急性髓性白血病（AML）的谱，对370例新诊断的CBF相关AML患者进行了分析。患者年龄16-83岁（中位39岁），男性略占优势（55%）；53%有静脉栓塞（16例）；47%为t（8;21）。t（8;21）的患者往往更年轻（P = 0.056），外周血白细胞计数较低（P < 0.0001），更容易出现额外的细胞遗传学异常（P < 0.0001）。性染色体缺失、del（9q）和复杂异常在t患者中更为常见（8;21），而+22和+21在inv患者中更为常见（16）。总体而言，87%[95%置信区间（CI） 83-90%]的患者达到完全缓解（CR）， t（8;21）和inv（16）之间无差异；然而，由于耐药疾病增加和早期死亡，老年患者的CR率较低。10年总生存率（OS）为44% (95% CI 39-50%)，在多变量分析中，随着年龄的增加（P < 0.0001），外周血母细胞百分比的增加（P = 0.0006），在复杂细胞遗传学异常和CBF易位的患者中（P = 0.021），以及t（8;21）患者中（P = 0.025）， 10年总生存率（OS）较短。在接受高剂量阿糖胞苷、氟达拉滨和中剂量阿糖胞苷联合治疗或造血细胞移植治疗的患者中，OS优于其他治疗方案。
To better understand the spectrum of adult acute myeloid leukaemia (AML) associated with core binding factor (CBF) translocations, 370 patients with newly diagnosed CBF-associated AML were analysed. Patients' age ranged from 16-83 years (median 39 years) with a slight male predominance (55%); 53% had inv(16); 47% had t(8;21). Patients with t(8;21) tended to be younger (P = 0.056), have lower peripheral blood white cell counts (P < 0.0001) and were more likely to have additional cytogenetic abnormalities (P < 0.0001). Loss of sex chromosome, del(9q) and complex abnormalities were more common among patients with t(8;21), while +22 and +21 were more common with inv(16). Overall, 87% [95% confidence interval (CI) 83-90%] of patients achieved complete response (CR) with no difference between t(8;21) and inv(16); however, the CR rate was lower in older patients due to increased resistant disease and early deaths. Ten-year overall survival (OS) was 44% (95% CI 39-50%) and, in multivariate analysis, was shorter with increasing age (P < 0.0001), increased peripheral blast percentage (P = 0.0006), in patients with complex cytogenetic abnormalities in addition to the CBF translocation (P = 0.021), and in patients with t(8;21) (P = 0.025). OS was superior in patients who received regimens with high-dose cytarabine, a combination of fludarabine and intermediate-dose cytarabine, or haematopoietic cell transplantation.