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目的观察重庆市多中心收治的67例FLT3-ITD~+AML患者中FLT3-ITD突变和NPM1、WT1等突变重叠在AML中的分布情况。方法总结FLT3-ITD~+AML患者的临床特点、疗效及异基因造血干细胞移植对其预后的影响。结果 FLT3-ITD~+AML免疫分型以M5型最多见,约占36%,其次为M2型,约占28%;该类患者常规化疗诱导缓解效果差,仅有27.5%的患者在第1次化疗达CR,且约46.3%的患者合并其他基因突变;在达CR1后尽早行HSCT有利于改善患者的不良预后,但移植相关死亡率及移植后的高复发风险仍是影响FLT3-ITD~+AML预后的重要因素。结论 FLT3-ITD突变是急性髓系白血病常见的突变类型,多数FLT3-ITD阳性患者具有外周血白细胞计数高、骨髓原始细胞比例高、预后差等特点;FLT3-ITD~+AML患者约有50%的患者合并其他基因突变。FLT3-ITD~+AML达CR1后尽早行异基因造血干细胞移植可有效改善FLT3-ITD~+AML患者的不良预后,HLA不全相合造血干细胞移植与HLA全相合造血干细胞移植疗效相似。
Objective To observe the distribution of FLT3-ITD mutations and NPM1, WT1 mutations overlapped in AML in 67 patients with FLT3-ITD ~ + AML treated in multicentre in Chongqing. Methods The clinical features, therapeutic effects and allogeneic hematopoietic stem cell transplantation on the prognosis of patients with FLT3-ITD ~ + AML were summarized. Results The most common type of FLT3-ITD ~ + AML was M5, accounting for 36%, followed by M2, accounting for 28%. The response to conventional chemotherapy in FLT3-ITD ~ + AML was poor. Only 27.5% Chemotherapy up to CR, and about 46.3% of patients with other genetic mutations; as soon as after CR1 line HSCT is conducive to improving the patient’s poor prognosis, but the transplant-related mortality and the high risk of recurrence after transplantation is still affect FLT3-ITD ~ + AML prognosis of the important factors. Conclusion FLT3-ITD mutation is a common mutation in acute myeloid leukemia. Most FLT3-ITD positive patients have the characteristics of high peripheral blood leukocyte count, high proportion of bone marrow blast cells and poor prognosis. FLT3-ITD ~ + AML patients have about 50% Of patients with other genetic mutations. Allogeneic hematopoietic stem cell transplantation after FLT3-ITD ~ + AML reaches CR1 can effectively improve the unfavorable prognosis of patients with FLT3-ITD ~ + AML. HLA-incompatible hematopoietic stem cell transplantation is similar to HLA-identical hematopoietic stem cell transplantation.