90例伴11q23/KMT2A异常原发急性髓系白血病的特征分析

Analysis of the characteristics of primary acute myeloid leukemia with 11q23/KMT2A rearrangements in ninety patients

目的探讨伴有11q23/KMT2A异常的原发性急性髓系白血病(AML)的临床及预后特征。方法回顾性分析90例伴有11q23/KMT2A异常的原发性AML患者的临床资料。结果在90例患者中,累及11q23的易位异常占80例,以t(9;11)(p22;q23)、t(6;11)(q27;q23)、t(10;11)(p12;q23)和t(11;19)(q23;p13)最为常见,非易位异常10例。完全缓解(CR)率为75.6%,携带t(6;11)者的CR率显著低于其他患者(47.1%vs.82.2%,P=0.005)。中位随访24.5个月,接受异基因造血干细胞移植(allo-HSCT)的患者的3年总体生存率(80.3%vs.16.6%,P<0.001)和3年无事件生存率(73.5%vs.16.3%,P<0.001)均显著优于非移植患者。T(6;11)者生存最差,3年总体生存率(11.8%vs.56.9%,P<0.001)和3年无事件生存率(5.9%vs.54.4%,P<0.001)均显著差于其他类型。无论是否接受allo-HSCT,t(9;11)和非t(9;11)患者的生存无统计学差异。结论伴11q23/KMT2A重排的原发性AML的临床特性具有异质性,未接受移植的患者预后更差,尤以t(6;11)的预后最差。Allo-HSCT可显著改善这类患者的生存。

Objective To investigate the clinical and prognostic characteristics of primary acute myeloid leukemia(AML)with 11q23/KMT2A rearrangements.Methods Clinical data of 90 patients with primary AML and 11q23/KMT2A rearrangements were analyzed retrospectively.Results By karyotyping analysis,80 of the 90 patients had translocations involving 11q23/KMT2A,with t(9;11)(p22;q23),t(6;11)(q27;q23),t(10;11)(p12;q23)and t(11;19)(q23;p13)being the most common ones,while 10 cases were found to have non-translocation abnormalities.The overall complete remission(CR)rate was 75.6%,and patients with t(6;11)had lower CR rate compared with non-t(6;11)patients(47.1%vs.82.2%,P=0.005).After a median follow-up of 24.5 months,the patients receiving allo-hematopoietic stem cell transplantation(allo-HSCT)had significantly higher 3-year overall survival(OS)(80.3%vs.16.6%,P<0.001)and 3-year event-free survival(EFS)(73.5%vs.16.3%,P<0.001)compared with non-transplant patients.Patients with t(6;11)had the lowest 3-year OS(11.8%vs.56.0%,P<0.001)and 3-year EFS(5.9%vs.53.8%,P<0.001)compared with other type of abnormalities.No significant difference was noted in the survival between patients with t(9;11)and non-t(9;11)regardless whether they had received HSCT.Conclusion The clinical characteristics of primary AML with 11q23/KMT2A rearrangements are heterogeneous.Patients did not receive HSCT had poorer survival,particularly with the presence of t(6;11).Allo-HSCT could significantly improve the survival of such patients.