290例成人急性髓系白血病临床特点及治疗效果

Clinical characteristics and treatment effect of acute myeloid leukemia in 290 adult patients

目的分析成人急性髓系白血病(AML)的临床特点、诊疗现状和远期疗效。方法收集西南医科大学附属医院血液内科住院治疗的290例成人AML患者临床资料,分析患者临床特征和预后差异。结果 290例的中位发病年龄为50(14~86)岁,男性153例,女性137例;有MDS病史者7例。按细胞遗传学预后分层,预后良好20例,预后中等84例,预后不良79例,无法分层107例,分别占6.9%、29.0%、27.2%、36.9%。分子学检测提示,FLT3-ITD、NPM1、c-KIT、AML-ETO基因异常分别占24.0%、17.0%、2.0%、11.2%;复杂核型(≥3种)占14.0%。55.9%患者因经济原因或疾病认识不足选择保守治疗。应用标准剂量DA/HA/IDA的2疗程诱导缓解率为70.5%,诱导相关死亡率为9.1%。缓解后应用含大剂量阿糖胞苷[Ara-C,≥2.0 g/(m^2·d),3 d]维持治疗的占28.4%,未使用含大剂量阿糖胞苷维持的占71.60%,其对应的中位无病生存时间(DFS)分别为12个月、7个月(P=0.098),预计3年总生存(OS)率分别为50.1%、23.7%(P=0.015)。结论本组AML患者性别、年龄、疾病亚型分布与国外稍有不同,但与国内多个中心的情况基本一致。标准缓解方案获得的缓解率处于国内中上水平,使用大剂量Ara-C维持治疗能获得更好的远期疗效。

Objective To analyze the clinical characteristics and current diagnosis and treatment of acute myeloid leukemia （AML） in adult patients and the long-term efficacy. Methods Clinical data of 290 adult patients with AML hospitalized in the Department of Hematopathology of the Affiliated Hospital of Southwest Medical University were collected and the clinical characteristics and difference in prognosis of these patients were analyzed. Results Among these 290 patients, the median age of onset was 50 （14-86）, there were 153 male patients and 137 female patients, and seven patients of them had a history of MDS. According to the cytogenetically prognostic stratification, there were 20 patients with favorable prognosis, 84 patients with moderate prognosis and 79 patients with unfavorable prognosis and 107 patients cannot be stratified, which accounted for 6.9%, 29.0%, 27.2% and 36.9% respectively. Molecular test suggested that the patients with abnormalities in genes FLT3-ITD, NPM1, c-KIT and AME-ETO accounted for 24.0%, 17.0%, 2.0% and 11.2% respectively; the patients with complex chromosomal abnormalities （at least three chromosomes） accounted for 14,0%; 55.9% patients chose conservative treatment for economic reason or short of knowledge about this disease. After two treatment courses with standard dose of DA/HA/IDA, the induced remission rate was 70.5% and the induction related mortality was 9.1%. After remission, the patients who received and who did not receive high-dose arabinosylcytosine [Ara-C, ≥ 2.0 g/（m·d）, 3 d] for maintenance treatment accounted for 28.4% and 71.6% respectively; the corresponding median disease-free survival （DFS） was 12 months and seven months respectively （P=0.098）; the corresponding 3-year overall survival （OS） rate was predicted to be 50.1% and 23.7% respectively （P=0.015）. Conclusion The gender, age and distribution of subtypes show slight differences between this group of AML patients and overseas groups, but are basically the same as those in multiple domestic centers. The remission rate of standard remission protocol is above the average in China; the maintenance treatment with high dose of Ara-C can achieve better long-term efficacy.