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异基因造血细胞移植可改善 t(6;9) 急性髓系白血病成人患者的预后:一项国际协作研究的结果。

Allogeneic hematopoietic cell transplantation improves outcome of adults with t(6;9) acute myeloid leukemia: results from an international collaborative study.

机构信息

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.

出版信息

Haematologica. 2020 Jan;105(1):161-169. doi: 10.3324/haematol.2018.208678. Epub 2019 Apr 19.

Abstract

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.

摘要

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。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/95cc/6939530/031c6c5a38ad/105161.fig1.jpg

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