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PICALM-MLLT10 急性髓系白血病:法国 18 例患者队列研究。

PICALM-MLLT10 acute myeloid leukemia: a French cohort of 18 patients.

机构信息

Service d'Hématologie, CHU de Toulouse, Hôpital Purpan, 31059 Toulouse, France.

出版信息

Leuk Res. 2012 Nov;36(11):1365-9. doi: 10.1016/j.leukres.2012.07.008. Epub 2012 Aug 5.

Abstract

The PICALM-MLLT10 fusion gene, generated by the t(10;11)(p12-13;q14-21) translocation, is a rare but recurrent event in acute leukemias. In this study, we assessed the characteristics and outcome of 18 PICALM-MLLT10 AML patients. As compared with non PICALM-MLLT10 patients (n=72), PICALM-MLLT10 AML were characterized by more frequent extramedullary diseases, CD7 expression and higher platelet counts. Three out of four therapy-related PICALM-MLLT10 AMLs had been previously treated for diffuse large B-cell lymphoma. The complete response rate was 71% after intensive chemotherapy. PICALM-MLLT10 patients had a shorter median overall survival than patients with favorable cytogenetics (12 months vs. not reached, p=0.07) but not significantly different from those of intermediate (26 months, p=0.32) or unfavorable cytogenetic groups (8 months, p=0.13). Long term responses were achieved in a subset of patients after allogeneic stem-cell transplantation but also after high-dose cytarabine.

摘要

PICALM-MLLT10 融合基因由 t(10;11)(p12-13;q14-21)易位产生,是急性白血病中一种罕见但反复出现的事件。在这项研究中,我们评估了 18 例 PICALM-MLLT10 AML 患者的特征和结果。与非 PICALM-MLLT10 患者(n=72)相比,PICALM-MLLT10 AML 更常伴有髓外疾病、CD7 表达和更高的血小板计数。4 例治疗相关 PICALM-MLLT10 AML 中有 3 例之前曾因弥漫性大 B 细胞淋巴瘤接受治疗。强化化疗后的完全缓解率为 71%。PICALM-MLLT10 患者的总生存期中位数短于具有良好细胞遗传学特征的患者(12 个月 vs. 未达到,p=0.07),但与中间组(26 个月,p=0.32)或不良细胞遗传学组(8 个月,p=0.13)无显著差异。在异基因造血干细胞移植后和高剂量阿糖胞苷治疗后,一部分患者实现了长期缓解。

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