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