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多中心回顾性分析采用急性髓系白血病样或急性淋巴细胞白血病样化疗方案治疗的成年混合表型急性白血病患者的临床结局及异基因干细胞移植的影响:一项校园ALL研究

Multicenter retrospective analysis of clinical outcome of adult patients with mixed-phenotype acute leukemia treated with acute myeloid leukemia-like or acute lymphoblastic leukemia-like chemotherapy and impact of allogeneic stem cell transplantation: a Campus ALL study.

作者信息

Lazzarotto Davide, Tanasi Ilaria, Vitale Antonella, Piccini Matteo, Dargenio Michelina, Giglio Fabio, Forghieri Fabio, Fracchiolla Nicola, Cerrano Marco, Todisco Elisabetta, Papayannidis Cristina, Leoncin Matteo, Defina Marzia, Guolo Fabio, Pasciolla Crescenza, Delia Mario, Chiusolo Patrizia, Mulè Antonino, Candoni Anna, Bonifacio Massimiliano, Pizzolo Giovanni, Foà Robin

机构信息

Clinica Ematologica-Centro Trapianti e Terapie Cellulari, Azienda Sanitaria Universitaria Friuli Centrale, Udine, Italy.

Dipartimento Di Medicina, Sezione Di Ematologia, Università Di Verona, Verona, Italy.

出版信息

Ann Hematol. 2023 May;102(5):1099-1109. doi: 10.1007/s00277-023-05162-0. Epub 2023 Mar 24.

Abstract

Mixed-phenotype acute leukemia (MPAL) is a rare disease. Treatment is often similar to that of acute lymphoblastic leukemia (ALL), but the outcome in adults and the role of allogeneic stem cell transplantation (AlloSCT) are not well defined. We report on 77 adult patients diagnosed with MPAL over the last 10 years and treated with a curative intent. Median age was 49 years; 7.6% of cases had a BCR::ABL1 rearrangement. Thirty patients (39%) were treated with an acute myeloid leukemia (AML)-like induction and 47 (61%) with an ALL-like scheme. The complete remission (CR) rate was 67.6% and an ALL-like therapy was associated with a better CR rate (P = 0.048). The median OS was 41.9 months; age ≤ 60 years was associated with a better OS (67 vs 26 months, P = 0.014). An AlloSCT was performed in 50 patients (65%). The 5-year OS of transplanted patients was 54%. The OS post-AlloSCT was better in patients who were minimal residual disease (MRD)-negative prior to transplant (75.8% vs 45.2%, P = 0.06). This study shows that MPAL patients respond better to an ALL-like induction therapy; that consolidation therapy should include, whenever possible, an AlloSCT and that MRD negativity should be a primary endpoint of treatment.

摘要

混合表型急性白血病(MPAL)是一种罕见疾病。其治疗通常与急性淋巴细胞白血病(ALL)相似,但成人患者的预后以及异基因干细胞移植(AlloSCT)的作用尚不明确。我们报告了过去10年中诊断为MPAL并接受根治性治疗的77例成年患者。中位年龄为49岁;7.6%的病例存在BCR::ABL1重排。30例患者(39%)接受了类似急性髓系白血病(AML)的诱导治疗,47例(61%)接受了类似ALL的方案治疗。完全缓解(CR)率为67.6%,类似ALL的治疗方案与更高的CR率相关(P = 0.048)。中位总生存期(OS)为41.9个月;年龄≤60岁与更好的OS相关(67个月对26个月,P = 0.014)。50例患者(65%)接受了AlloSCT。移植患者的5年OS率为54%。移植前微小残留病(MRD)阴性的患者AlloSCT后的OS更好(75.8%对45.2%,P = 0.06)。这项研究表明,MPAL患者对类似ALL的诱导治疗反应更好;巩固治疗应尽可能包括AlloSCT,且MRD阴性应作为治疗的主要终点。

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