核结合因子相关的急性髓系白血病的临床分析

Clinical and Cytogenetic Features and Their Influencing Factors of Core Binding Factor Acute Myeloid Leukemia

目的探讨影响核结合因子(CBF)相关的急性髓系白血病(AML)患者临床特征、细胞遗传学特点及影响其生存、预后的主要因素。方法对130例CBF AML[其中AML伴t(8;21)87例、AML伴inv(16)/t(16;16)43例]患者进行随访,分析其免疫表型、染色体核型及治疗、生存情况、影响总体生存时间及无复发生存时间的因素。结果 130例CBF AML患者总完全缓解率96.1%,其中1疗程总完全缓解率77.2%。中位生存期(OS)51.64(0.26～132.5)个月,中位无复发生存期(RFS)未达1.18～96.62个月。3年OS率50%,5年OS率41%;3年RFS率59%,5年RFS率54%。年龄、染色体核型与OS有关,其中年龄≥45岁患者、染色体核型伴9q-的患者预后差、生存期短。在巩固治疗过程中采用≥2疗程的中剂量阿糖胞苷(Ara-C)巩固治疗方案的患者预后好,生存期长。OS、RFS对比分析显示:AML伴inv(16)/t(16;16)的OS较AML伴t(8,21)明显延长(P=0.046),但AML伴t(8,21)的RFS较AML伴inv(16)/t(16;16)明显延长(P=0.038)。结论年龄、染色体核型及巩固治疗的方案是影响CBFAML患者生存、预后的主要因素,在巩固治疗中采用≥2疗程的中剂量Ara-C可以延长CBF AML患者的OS、RFS。AML伴inv(16)/t(16;16)患者较AML伴t(8,21)患者总体生存期长、预后好。

Objective To discuss the clinical and cytogenetic features of core binding factor（CBF） acute myeloid leukemia（AML） patients and the main factors that influence the prognosis.Method Totally 130 CBF AML patients were followed up and their clinical features,immunophenotype,chromosome karyotype,treatment regimen,overall survival（OS）,and relapse-free survival（RFS）were analyzed.Results The overall complete remission（CR） rate was 96.1%,among which the CR rate after the first treatment course was 77.2%.The overall median OS was 51.64（0.26-132.5） months,while the median RFS did not reach 1.18-96.62 months.The 3-year OS was 50% and the 5-year OS was 41%;the 3-year RFS was 59% and the 5-year RFS was 54%.Patients who were over 45 years and those with chromsome karyotype of 9q-tended to have poorer prognosis.During the consolidating chemotherapy,patients who had received two or more courses of intermediate-dose Ara-C therapy had better prognosis and longer survival.AML patients with inv（16）/t（16;16） had a significantly higher OS than those with t（8;21）（P=0.046）,while the RFS showed an opposite finding（P=0.038）.Conclusions Age,chromosomal karyocyte,and consolidating chemotherapy are the main factors that influence the survival and prognosis of CBF AML patients.Two or more courses of intermediate-dose Ara-C during consolidating chemotherapy can obviously prolong the OS and RFS of CBF AML patients.AML patients with a chromosomal karyocyte of inv（16）/t（16;16） have longer OS and better prognosis than those with t（8;21）.