中文摘要：

目的探讨动态监测RUNX1-RUNX1T1转录本水平在儿童t（8；21）急性髓系白血病（AML）预后评估中的价值。方法以2010年1月至2016年4月就诊于郑州大学人民医院的55例儿童t（8；21）AML患者为研究对象，应用实时荧光定量PCR（RQ-PCR）动态监测患者的RUNX1-RUNX1T1转录本水平，分析其变化与疾病预后的关系。结果初诊时患者骨髓细胞中的RUNX1-RUNX1T1转录本水平与复发及预后无关。1个疗程诱导缓解治疗后，骨髓细胞中RUNX1-RUNX1T1转录本水平比诊断时下降大于2个对数级（a〉2Log）者与a≤2Log者相比：5年累积复发率（CIR）分别为（24．3±8．4）％及（52．6±9．7）％（x^2=9．046，P=-0．003），5年无复发生存（RFs）率分别为（71．6±12．7）％及（48．1±13．2）％（x^2=5．814，P=0．016），5年总生存（OS）率分别为（76．9±12．5）％及（48．9±14．7）％（x^2=6．346，P=0．012），差异均有统计学意义。多因素Cox回归模型结果显示，1个疗程诱导缓解后RUNX1-RUNX1T1转录本下降是否大于2Log是影响RFS（HR=0．263，95％CI0．081～0．851，P=0．026）与OS（HR=0．214，95％C10．057-0．808，P=0．023）的独立预后因素。巩固治疗及治疗结束随访期间动态监测RUNX1-RUNX1T1转录本水平变化，共有16例患者发生分子学复发，其中13例患者发生了血液学复发，分子学复发至血液学复发的中位时间为4．0（1．5-5．8）个月。对2例分子学复发后的患者及时采取异基因造血干细胞移植治疗后，患者未发生血液学复发。结论通过RQ—PCR动态监测儿童t（8；21）AML患者的RUNX1-RUNX1T1转录本水平，可以将儿童t（8；21）AML细分为相对低危组和相对高危组并提前预测血液学复发，为实现儿童t（8；21）AML的精准分层和风险一适应治疗提供科学依据。

英文摘要：

Objective To investigate the prognostic value of dynamic monitoring of RUNX1- RUNX1T1 transcript in pediatric patients with t（8;21） acute myeloid leukemia （AML）. Methods The clinical features and RUNX1-RUNX1T1 transcript levels of 55 pediatric t （8;21） AML patients, newly diagnosed from Jan. 2010 to Apr. 2016, were analyzed retrospectively. The relationship between the minimal residual disease （MRD） and prognosis was analysed by dynamic monitoring of RUNX1- RUNX1T1 transcript levels using real-time quantitative PCR （RQ-PCR） technology. Results The RUNX 1-RUNX 1T 1 transcript levels in bone marrow cells at diagnosis was not related to relapse. After one course of induction therapy, patients with a more than 2 Log reduction of RUNX1-RUNX1T1 transcript levels （〉2 Log） had lower 5 years cumulative incidence of relapse （CIR） [ （24.3±8.4）% vs （52.6±9.7）%, x^2=9.046, P=0.003], relapse-free survival （RFS） [ （71.6±12.7）% vs （48.1±13.2）%,x^2=5.814, P=-0.016], and better overall survival （OS） [ （76.9±12.5）% vs （48.9± 14.7）%, x^2=6.346, P=0.012], compared to patients with a less than 2 Log reduction （a〈2 Log）. Multivariate Cox survival analysis suggested that a〉2Log reduction in RUNX1-RUNX1T1 transcript levels after a course of induction therapy was an independent prognostic factor for RFS （HR=0.263, 95%CI 0.081-0.851, P=0.026） and OS （HR=0.214, 95% CI 0.057-0.808, P=0.023）. During consolidation therapy and follow-up period, molecular relapse of 16 cases and hematologic relapse of 13 cases were identified by continuous dynamic monitoring of RUNX1- RUNX 1T 1 transcript levels, with a median interval of 4.0 （ 1.5- 5.8 ） months from the molecular relapse to hematologic relapse. 2 cases of molecular relapse who received timely allogeneic hematopoietic stem cell transplantation did not experience hematologic relapse. Conclusion Dynamic monitoring RUNX1- RUNX1T1 transcript levels by RQ-PCR technique can subdivide patients into relatively low an