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摘要: 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。
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关键词:
- AML1/ETO阳性AML /
- 疾病特征 /
- 治疗预后
Abstract: Objective:To analyze the clinical and molecular characteristics of acute leukemia with AML1/ETO and explore the reasonable therapeutic principles.Method:Characteristics in morphology, immunology, molecular biology, cytogenetics, treatment and overall survival in 83 cases of acute leukemia with AML1/ETO were studied and analyzed. Result:Within 83 cases of acute leukemia with AML1/ETO, 17 cases(20.4%) had extramedullary infiltration, 47 cases(57.8%) with WBC count greater than 10×109/L, 45 cases(60.8%) with CD56 antigen positive, 29 cases(39.2%) with CD19 antigen positive. 65 patients had t(8;21) translocation which included 28 cases of t(8;21)translocation alone(43.1%), 37 cases(56.9%) with additional chromosome abnormality. All the 83 patients received induction chemotherapy and the overall CR rate was 79.5%, 17 patients relapsed within 6 months after CR in 66 patients who had achieved CR and the relapse rate was 25.9%. In 83 cases, 42 cases survived and 41 cases died, the median survival time was 26(range 2-113) months. 36 cases received allo-hematopoietic stem cell transplantation(HSCT), 25 cases survived and 3-year overall survival rate was 67.5%, 5-year overall survival rate was 56.6%. The 3-year overall survival rate was 36.4% in 47 patients who received chemotherapy. The analysis of prognosis factors showed that the patients’age and whether they express CD56 antigen or not had no statistical effect on overall survival, but the WBC count at the presentation, extramedullary infiltration, additional chromosome abnormality and treatment regimen (chemotherapy or transplantation) had statistical effects on overall survival. The overall survival in patients after HSCT was much higher than that in patients who received chemotherapy(P<0.01).Conclusion:Acute leukemia patients with AML1/ETO would be prone to accompanied with high-risk factors, and HSCT could obviously improve the survival conditions of these patients. -
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