中文摘要：

目的 探讨FLT3-ITD突变阳性急性髓系白血病（AML）的临床特征和预后因素,为FLT3-ITD突变阳性AML患者的预后评估提供更多证据.方法 收集98例FLT3-ITD突变阳性AML初诊患者的临床资料,对其临床特征与预后的相关性进行统计学分析.结果 98例FLT3-ITD突变阳性AML患者初诊时中位WBC 58.2 （0.3～ 461.8）×10^9/L,外周血原始细胞绝对计数中位数42.2（0～397.1）×10^9/L.71例细胞遗传学中高危组患者1个疗程完全缓解（CR）率为60.6％,原发耐药率为26.8％.单因素分析结果显示外周血原始细胞绝对计数低是影响患者1个疗程CR的良好预后因素（P=0.009）.多因素分析结果显示,1个疗程达CR （HR=0.395,95％ CI 0.183～0.850,P=0.001）及第1次CR期行异基因造血干细胞移植（HR=0.180,95％CI 0.043～0.752,P=0.018）是影响患者无复发生存的良好预后因素,1个疗程达CR（HR=0.251,95％CI 0.121～0.523,P＜0.001）及外周血原始细胞绝对计数低（HR=0.219,95％CI 0.088～0.545,P=0.003）是影响患者总生存的良好预后因素.结论 外周血原始细胞计数低的FLT3-ITD突变阳性AML患者预后相对良好,1个疗程达CR和第1次CR期行异基因造血干细胞移植是改善FLT3-ITD突变阳性AML患者预后的重要治疗策略.

英文摘要：

Objective To investigate the clinical significance and prognosis factors of acute myeloid leukemia （AML） with FLT3 mutation-internal tandem duplication （FLT3-ITD） and provide more evidence for prognosis evaluation in AML with FLT3-ITD.Methods The clinical characteristics and outcomes of 98 AML with FLT3-ITD were analyzed.Results In patients with FLT3-ITD positive AML,the median of WBC and peripheral blood blast at initial diagnosis were 58.2 （0.3-461.8）× 10^9/L and 42.2 （0-397.1） × 10^9/L,respectively.The complete remission （CR） rate after one course induction therapy was 60.6% in 71 patients with intermediate or adverse risk.The primary refractory rate was 26.8% in 71 patients with intermediate or adverse risk.The lower number of peripheral blood blast was a favorable factor to achieve CR after one course induction therapy（P=0.009）.CR after one course （HR=0.395,95% CI 0.183-0.85,P=0.001） and allo-transplantation in CR1 （HR=0.180,95% CI 0.043-0.752,P=0.018） favored longer relapse-free survival （RFS）.CR after one course （HR=0.25 1,95% CI 0.121-0.523,P〈 0.001） and lower number of peripheral blood blast （HR=0.219,95% CI 0.088-0.545,P=0.003） favored longer overall survival （OS） in AML with FLT3-ITD.Conclusion FLT3-ITD positive AML with lower number of peripheral blood blast has a relative favorable prognosis.CR after one course and allotransplantation in CR1 are important strategy to improve prognosis in AML with FLT3-ITD.