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目的:分析AML1/ETO阳性急性髓系白血病(AML)患者的分子生物学和临床特点,探讨治疗策略。方法:对83例AML1/ETO阳性的AML患者的形态学、免疫学、分子生物学、染色体特征以及治疗、生存情况进行分析总结。结果:83例AML1/ETO阳性的AML患者中,髓外浸润17例(20.4%),白细胞大于10×109/L的47例(57.8%);45例患者表达CD56抗原(60.8%),29例表达CD19抗原(39.2%);有65例患者检测到t(8;21)异位,其中包括单独t(8;21)异位的28例(43.1%)、伴有附加染色体的37例(56.9%);83例患者均进行了诱导化疗,总缓解率为79.5%,66例缓解后的患者有17例在6个月内复发,复发率为25.9%;83例患者共存活42例,死亡41例,中位生存期为26(2~113)个月;有36例进行了异基因造血干细胞移植(HSCT),25例存活,3年OS率67.5%,5年OS是56.6%,另外47例接受化疗的患者3年OS率36.4%;预后因素分析显示,患者的年龄、CD56的表达对OS率的影响差异无统计学意义,而初诊时白细胞数目、髓外浸润、有无附加染色体以及治疗的情况(化疗和移植)对OS率的影响差异有统计学意义;采用异基因造血干细胞移植的患者的OS率明显高于接受化疗的患者(P<0.01)。结论:AML1/ETO阳性AML患者易并发高危因素,异基因造血干细胞移植可明显改善AML1/ETO阳性AML患者的生存。
Objective: To analyze the molecular biology and clinical features of patients with AML1 / ETO-positive acute myeloid leukemia (AML) and to explore the therapeutic strategies. Methods: The morphology, immunology, molecular biology, chromosomal characteristics and treatment and survival of 83 AML1 / ETO positive AML patients were analyzed and summarized. Results: Among the 83 AML1 / ETO positive AML patients, extramedullary infiltration was found in 17 (20.4%) and white blood cells in 47 (57.8%) of those with more than 10 × 109 / L; 45 patients expressed CD56 antigen (60.8%), Cases expressed CD19 antigen (39.2%); 65 patients had ectopic t (8; 21) detected including 28 (43.1%) cases of ectopic t (8; 21) alone, 37 cases with additional chromosome (56.9%). All of the 83 patients underwent induction chemotherapy with a total response rate of 79.5%. Seventeen patients relapsed within 6 months with a relapse rate of 25.9% in 66 patients with remission, and 83 patients had 42 patients , 41 died, the median survival was 26 (2 ~ 113) months; 36 cases were allogeneic hematopoietic stem cell transplantation (HSCT), 25 patients survived, 3-year OS rate 67.5%, 5-year OS was 56.6% , And another 47 patients received chemotherapy in 3-year OS rate of 36.4%; prognostic factor analysis showed that the patient’s age, CD56 expression on OS rate was no significant difference, but the number of leukocytes at initial diagnosis, extramedullary infiltration, with or without The effect of additional chromosomes and treatment (chemotherapy and transplantation) on OS rates was significantly different; patients with allogeneic hematopoietic stem cell transplantation had significantly higher OS rates than those treated with chemotherapy (P <0.01). Conclusion: Patients with AML1 / ETO positive AML are prone to be complicated by high risk factors. Allogeneic hematopoietic stem cell transplantation can significantly improve the survival of AML1 / ETO positive AML patients.