诱导化疗后骨髓缓解程度对儿童核心结合因子相关性急性髓系白血病预后的影响

The Impact of Induction Treatment Response on the Prognosis of Pediatric Core Binding Factor-Acute Myeloid Leukemia Patients

目的:探讨诱导化疗后骨髓形态学缓解程度对儿童核心结合因子相关性急性髓系白血病(CBF-AML)预后的影响。方法:回顾性分析2008年9月至2018年12月我科收治的157例CBF-AML患儿诱导后反应结果及生存资料。对不同骨髓缓解程度患儿的生存进行对比分析。结果:157例患者中,113例(72.4%)第一个疗程诱导化疗后获形态学无白血病状态(MLFS),153例(98.1%)在第二个疗程后获MLFS。1个疗程诱导后未缓解(NR)患儿的5年无事件生存(EFS)率及总生存(OS)率均显著性低于1个疗程诱导化疗后获MLFS及部分缓解(PR)的患儿。经过2个疗程诱导化疗后达到PR患儿的5年EFS及OS均明显低于达到MLFS的患儿,但差异无统计学意义。多因素分析显示,1个疗程NR及伴髓系肉瘤为影响CBF-AML患儿EFS的独立危险因素。本研究中,共6例患儿1个疗程后NR均为伴t(8;21)患儿其中有3例伴性染色体缺失,有2例伴髓系肉瘤。结论:1个疗程诱导化疗后形态学NR为影响CBF-AML患儿EFS及OS的独立危险因素应提高危险度及早调整治疗方案以改善预后。对于CBF-A ML患儿来说,诱导化疗PR患儿可不作为原发耐药的诊断标准。

Objective:To explore the impact of induction treatment response on the prognosis of pediatric core binding factor-acute myeloid leukemia(CBF-AML).Methods:The result of induce reaction and survival data of 157 pediatric CBF-AML patients in our hospital from September 2008 to December 2018 were retrospectively analyzed.The survival rate of the patients with different degrees of morphological remission after induction chemotherapy was comparative analyzed.Results:Among the 157 children with CBF-AML,113(72.4%)patients achieved morphologic leukemia-free state(MLFS)after the first course of induction chemotherapy,153(98.1%)patients achieved MLFS after the second course of induction chemotherapy.The 5-year event-free survival(EFS)rate and 5-year overall survival(OS)rate of patients with non-remission(NR)status after the first course of induction of chemotherapy was significantly lower than the patients achieved MLFS and the patients achieved partial remission(PR).The 5-year EFS rate and 5-year OS rate of the patients with PR status after the second course of induction chemotherapy were lower than the patients achieved MLFS,but the difference was not statistically significant.Multivariable analyze showed that NR after the first course of induction chemotherapy and myeloid sarcoma were the independent risk factors affecting EFS of the patients.There were six patients with NR status after the first course of induction chemotherapy,in which all of them harbored t(8;21),three of them with sex chromosome deletion,two of them with myeloid sarcoma.Conclusion:NR status after the first course of induction chemotherapy was the independent risk factor affecting EFS and OS of CBF-AML patients,it can be taken as an indicator for higher risk stratification.PR status after the first course of induction chemotherapy may not be used as a diagnostic criterion for primary drug resistance.