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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 investigate the clinical characteristics, cytogenetic characteristics and the main factors influencing the survival and prognosis of patients with acute myeloid leukemia (AML) related to nuclear factor-kappaB (CBF). Methods A total of 130 CBF AML patients (87 with AML with t (8; 21) and 43 with AML with inv (16) / t (16; 16) were followed up and analyzed for their immunophenotype, chromosomal karyotype and treatment , Survival, factors affecting the overall survival time and recurrence-free survival time. Results The total complete remission rate of 130 CBF AML patients was 96.1%, of which 1 course of complete remission rate was 77.2%. The median survival time (OS) was 51.64 (0.26 ~ 132.5) months, and the median relapse-free survival (RFS) was less than 1.18 ~ 96.62 months. 3-year OS rate of 50%, 5-year OS rate of 41%; 3-year RFS rate of 59%, 5-year RFS rate of 54%. Age, chromosomal karyotype and OS, in which patients aged 45 years or older, chromosome karyotype with 9q-poor prognosis of patients with short survival. Patients undergoing consolidation therapy with mid-dose cytarabine (Ara-C) at ≥2 cycles during consolidation therapy have a good prognosis and long survival. OS and RFS showed that the OS of AML with inv (16) / t (16; 16) was significantly longer than that of AML with t (8,21) (P = 0.046) Was significantly longer than that of AML with inv (16) / t (16; 16) (P = 0.038). Conclusion Age, chromosomal karyotype and consolidation therapy are the main factors influencing the survival and prognosis of patients with CBFAML. The mid-dose Ara-C of ≥2 courses in consolidation therapy can prolong the OS and RFS of CBF AML patients. Patients with AML with inv (16) / t (16; 16) had longer overall survival and better prognosis than those with AML (t = 8,21).