中国癌症防治杂志2023，Vol.15Issue(6)：637-643,7.DOI:10.3969/j.issn.1674-5671.2023.06.07
单中心CCLG-ALL 2018方案治疗MLL基因重排阳性儿童急性淋巴细胞白血病的临床研究
Clinical study of single-center CCLG-ALL 2018 regimen in the treatment of MLL-rearrangement childhood acute lymphoblastic leukemia
摘要
Abstract
Objective To investigate the efficacy and prognostic factors of single-center CCLG-ALL 2018 regimen in the treatment of children with MLL-rearrangement acute lymphoblastic leukemia(MLL-r ALL).Methods A total of 19 newly diagnosed children with MLL-r ALL who received CCLG-ALL 2018 regimen in Children's Hospital Affiliated to Zhengzhou University from April 2018 to July 2022 were retrospectively analyzed.Results Among the 19 children with MLL-r ALL,12(63.2%)were males and 7(36.8%)were females;15(78.9%)aged≤1 year old,and 4(21.1%)aged>1 year old.The median age at diagnosis was 10.2 months(range:0.5-156.0 months).There were 18(94.7%)cases of BCP-ALLand 1(5.3%)case of T-ALL.MLL-AF4was the most common fusion genotype(7/19).The median follow-up time was 19.0 months(range:0.1-62.0 months),and 1 patient died 5 days after diagnosis,with a complete response rate of 61.1%on the 33rd day of induction chemotherapy.In total,9 of 19 children died(47.4%),including 3 cases of treatment-related complications(1 case of pulmonary hemorrhage and 2 cases of severe infection).1 case died of leukemia without remission,5 cases died of recurrence with a recurrence rate of 26.3%(5/19),and all these cases were early bone marrow recurrence,with a median recurrence time of 6 months(range:5-9 months).Kaplan-Meier showed that the 2-year overall survival(OS)and event-free survival(EFS)rates of 19 children were 57.9%and 52.6%,respectively.The results of univariable analysis showed that there were significant differences in 2-year OS rate between the<1×10-3 group and≥1×10-3 group on the 15th day of minimal residual disease(MRD),between the MLL rearrangement gene negative and non-negative groups on the 33rd day,and between the MLL-AF4 group and the non-MLL-AF4 group(all P<0.05).Multivariable analysis of Cox model showed that MLL-AF4 was an independent adverse prognostic factor affecting the OS in children with MLL-r ALL(P=0.032).Conclusions CCLG-ALL 2018 regimen could achieve remission in some of children with MLL-rearrangement,but the overall prognosis is poor,prone to recurrence and high mortality rate at recurrence.The MLL-AF4 rearrangement is the adverse factor affecting the treatment effect of CCLG-ALL 2018 regimen in children with MLL-r ALL.关键词
急性淋巴细胞白血病/MLL基因重排/儿童/疗效/预后因素Key words
Acute lymphoblastic leukemia/MLL-rearrangement/Children/Curative effect/Prognostic factor分类
临床医学引用本文复制引用
李晨,刘炜,王亚峰,王天有..单中心CCLG-ALL 2018方案治疗MLL基因重排阳性儿童急性淋巴细胞白血病的临床研究[J].中国癌症防治杂志,2023,15(6):637-643,7.基金项目
河南省科技攻关项目(222102310616) （222102310616）
