Department of Internal Medicine V, University Hospital of Heidelberg, Heidelberg, Germany
German Cancer Research Center (DKFZ) and Department of Internal Medicine V, University of Heidelberg, Heidelberg, Germany.
Haematologica. 2020 Jan;105(1):161-169. doi: 10.3324/haematol.2018.208678. Epub 2019 Apr 19.
Acute myeloid leukemia (AML) with t(6;9)(p22;q34) is a distinct entity accounting for 1-2% of AML cases. A substantial proportion of these patients have a concomitant -ITD. While outcomes are dismal with intensive chemotherapy, limited evidence suggests allogeneic hematopoietic cell transplantation (allo-HCT) may improve survival if performed early during first complete remission. We report on a cohort of 178 patients with t(6;9)(p22;q34) within an international, multicenter collaboration. Median age was 46 years (range: 16-76), AML was in 88%, -ITD was present in 62%, and additional cytogenetic abnormalities in 21%. Complete remission was achieved in 81% (n=144), including 14 patients who received high-dose cytarabine after initial induction failure. With a median follow up of 5.43 years, estimated overall survival at five years was 38% (95%CI: 31-47%). Allo-HCT was performed in 117 (66%) patients, including 89 in first complete remission. Allo-HCT in first complete remission was associated with higher 5-year relapse-free and overall survival as compared to consolidation chemotherapy: 45% (95%CI: 35-59%) and 53% (95%CI: 42-66%) 7% (95%CI: 3-19%) and 23% (95%CI: 13-38%), respectively. For patients undergoing allo-HCT, there was no difference in overall survival rates at five years according to whether it was performed in first [53% (95%CI: 42-66%)], or second [58% (95%CI: 31-100%); n=10] complete remission or with active disease/relapse [54% (95%CI: 34-84%); n=18] (=0.67). Neither -ITD nor additional chromosomal abnormalities impacted survival. In conclusion, outcomes of t(6;9)(p22;q34) AML are poor with chemotherapy, and can be substantially improved with allo-HCT.
伴 t(6;9)(p22;q34) 的急性髓系白血病(AML)占 AML 病例的 1-2%,属于一种独特的疾病实体。这些患者中有相当一部分同时存在-ITD。尽管强化化疗的预后较差,但有限的证据表明,如果在首次完全缓解期早期进行同种异体造血细胞移植(allo-HCT),可能会改善生存。我们报告了一项国际多中心合作中伴 t(6;9)(p22;q34)的 178 例患者队列。中位年龄为 46 岁(范围:16-76 岁),AML 占 88%,-ITD 占 62%,其他细胞遗传学异常占 21%。81%(n=144)的患者达到完全缓解,包括 14 例初始诱导失败后接受高剂量阿糖胞苷治疗的患者。中位随访 5.43 年后,五年总生存率估计为 38%(95%CI:31-47%)。117 例(66%)患者接受了 allo-HCT,其中 89 例在首次完全缓解期。与巩固化疗相比,首次完全缓解期进行 allo-HCT 与较高的五年无复发生存率和总生存率相关:45%(95%CI:35-59%)和 53%(95%CI:42-66%),7%(95%CI:3-19%)和 23%(95%CI:13-38%)。对于接受 allo-HCT 的患者,五年总生存率无差异,无论allo-HCT 是在首次完全缓解期(53%[95%CI:42-66%])、第二次完全缓解期(58%[95%CI:31-100%];n=10)还是活动期/复发期(54%[95%CI:34-84%];n=18)进行(=0.67)。-ITD 或其他染色体异常均不影响生存。总之,伴 t(6;9)(p22;q34)的 AML 患者化疗预后较差,allo-HCT 可显著改善其预后。