急性早幼粒细胞白血病临床特点及生存相关因素分析

The clinical features of 202 cases with acute promyelocytic leukemia and the analysis of related elements

目的总结全反式维甲酸(ATRA)治疗急性早幼粒细胞白血病(APL)临床资料,探讨影响其长期生存的相关因素。方法采用临床病例分析方法。ATRA诱导缓解治疗急性早幼粒细胞白血病202例。缓解后按所接受的维持治疗分为维甲酸加化疗序贯交替组53例,单用化疗组50例,终止治疗组21例。观察长期生存率与年龄、性别、初诊时血液学指标的关系。结果202例患者中20例在接受ATRA治疗2周内死亡,早期病死率9·9%。182例患者中166例获得完全缓解(CR),缓解率91·2%。3年生存率40·4%,5年以上生存率22·6%(28/124)。APL合并中枢神经系统(CNS)浸润发生率为7·4%(15/202)。长期生存患者年龄在35岁以下者占85·7%。复发61例,复发率49·2%。结论年龄、初诊时白细胞数、白血病细胞浸润程度和合并CNS浸润是影响APL患者长期生存的重要因素。PML/RARα融合基因持续阳性或由阴性转为阳性常与复发有关,而持续阴性常伴随长期无病生存。

Objective To sum up the clinical data of treating acute promyelocytic leukemia（APL） with alltrans retinoic acid（ATRA） to research the long-term survival related elements. Methods The clinical eases were analyzed. 202 APL patients were treated with ATRA for induction remission. After the complete remission, they were divided into three groups in accord with their accepted maintenance therapies： （1）53 cases treated with ATRA and chemotherapy alternatively; （2）50 cases treated with chemotherapy only;（3） 21 cases without treatment. Observed the relation between long-term survival and age,sex, the criteria of hemotology at the first consultant. Results Of the 202 patients,20 treated with ATRA died in two weeks and the early fatality rates was 9.9%. Of the rest 182 patients, 166 got CR. The remission rate was 91.2%. Three-year survival was 40.4% , over five-year survival was 22.6% （28/124 ）. The rate of APL combined with CNS infiltration was 7.4%（15/202）. 85.7% long-term survival patients were under 35 years old. There were 61 relapsed cases. The relapse rate was 49.2%. Conclusions Of the long-term survival related elements, the age, the number of leukocyte at the first consultation,the degree of leukemia cell infiltration are of great importance to influence the long-term survival of APL patients. What PML/RARα fused gene remaine negative or turne from negative to positive often relate to relapse, and remaine negative often go with long-term survival without diseases.