混合表型急性白血病82例临床特点及疗效预后分析

Biological characteristics,treatment and outcomes of mixed phenotype acute leukemia

目的分析混合表型急性白血病(mixed phenotype acute leukemia,MPAL)的生物学特点、治疗及预后。方法依据1998年欧洲白血病免疫分型小组(EGIL)或2008年WHO血液肿瘤诊断标准,回顾性分析我中心82例双表型急性白血病(biphenotypic acute leukaemia,BAL)或MPAL患者资料,可进行疗效分析者75例,分析不同诱导及巩固治疗方案的疗效,从而探讨影响该类患者预后的相关因素。结果 MPAL患者中兼顾淋系和髓系的混合样方案完全缓解率为66.7%(20/30),急淋样方案完全缓解率为51.9%(14/27),急髓样方案完全缓解率为16.7%(3/18),差异有统计学意义(P=0.003),中位总生存期分别为47、21、16个月,3年的总生存(OS)率分别为(59.8±10.0)%、(41.2±10.4)%、(38.8±12.5)%,差异无统计学意义(P=0.239)。45例诱导化疗后接受造血干细胞移植治疗,其余30例则接受单纯化疗,移植组与单纯化疗组3年的OS率分别为(61.7±7.6)%、(22.3±9.4)%,差异有统计学意义(P=0.002),3年的无事件生存(EFS)率分别为(59.7±7.6)%、(19.3±8.4)%,差异有统计学意义(P=0.001)。结论采用急淋样方案或淋系、髓系的混合样方案可提高其缓解率,造血干细胞移植可以提高MPAL患者的远期生存。

Objective To analyze the biological characteristics,treatment and outcomes of mixed phenotype acuteleukemia(MPAL). Methods According to the 1998 EGIL or 2008 WHO hematologic tumor diagnostic criteria,we retrospectively analyzed 82 patients with Biphenotypic acute leukaemia(BAL)or MPAL data in our center,and75 patients can be enrolled with efficacy analysis. To compare the effectiveness of different induction and consolidation therapies,and further to explore the relevant factors affecting the outcomes of these patients. Results Among MPAL patients complete remission(CR)of patients received hybrid induction was 66.7%(20/30),the CR rate of patients treated with ALL like induction was 51.9%(14/27),and the CR rate of patients treated with AML like induction was 16.7%(3/18)(P = 0.003).The median OS durations in the hybrid induction,ALL-like induction,and AML-like induction therapy groups were 47 months[3 year OS(59.8 ± 10.0)%],20 months[3 year OS(39.8±10.2)%],and 16 months[3 year OS(34.2 ± 11.5)%],respectively(P = 0.239). 45 patients received the treatment of stem cell transplantation,while the other 30 patients received the chemotherapy alone after induction chemotherapy.The 3 year OS rates were(61.7 ± 7.6)% and(22.3 ± 9.4)% respectively(P = 0.002). The 3 year EFS was(59.7 ± 7.6)% and(19.3 ± 8.4)% respectively(P = 0.001). Conclusions ALL like induction or hybrid induction therapy is associated with a greater CR rate. Allogeneic hematopoietic stem cell transplantation(Allo-HSCT)can significantly improve the long-term survival of patients with MPAL.