临床儿科杂志 ›› 2017, Vol. 35 ›› Issue (5): 325-.doi: 10.3969/j.issn.1000-3606.2017.05.002

• 血液肿瘤疾病专栏 • 上一篇    下一篇

TEL/AML1 基因阳性急性淋巴细胞白血病患儿CCLG-ALL-2008方案疗效分析

高静, 胡绍燕, 卢俊, 何海龙, 王易, 赵文理, 李建琴, 李捷, 肖佩芳, 范俊杰, 柴忆欢   

  1. 苏州大学附属儿童医院血液科 ( 江苏苏州 215000 )
  • 收稿日期:2017-05-15 出版日期:2017-05-15 发布日期:2017-05-15
  • 通讯作者: 胡绍燕,肖佩芳 E-mail:hsy 139@126 .com; xpfdr@163 .com
  • 基金资助:
    国家自然科学基金项目(No.NSCF81170513和81370627);江苏省重点专病项目(No.BL2013014); 苏州市儿童白血病重点实验室(No.LCZX201507);苏州市小儿内科临床医学中心(No.SZZX201504); 苏州市临床重点专病(No.SZS201615)

Effect of chemotherapy regimen CCLG-ALL-2008 on children with TEL/AML1 fusion gene positive of acute lymphoblastic leukemia

 GAO Jing,HU Shaoyan,LU Jun,HE Hailong,WANG Yi,ZHAO Wenli,LI Jianqin,LI Jie,XIAO Peifang,FAN Junjie,CHAI Yihuan   

  1. Department of Hematology,Children’s Hospital of Soochow University,Suzhou 215000, Jiangsu,China
  • Received:2017-05-15 Online:2017-05-15 Published:2017-05-15

摘要: 目的 探讨CCLG-ALL-2008方案治疗TEL/AML1融合基因阳性儿童急性淋巴细胞白血病(ALL)效果以及影 响因素。方法 回顾分析2008年6月至2014年12月初诊并治疗的99例TEL/AML1融合基因阳性患儿及329例无特异性 融合基因的B系ALL患儿的临床和生物学特征,随访至2015年10月,观察两组患儿的生存状态和影响因素。结果 两组 患儿初诊时的年龄和外周血白细胞数相近;在诱导缓解治疗第8天的泼尼松敏感实验、第15天骨髓呈M3状态、第33天 和第12周微小残留病灶(MRD)>1.0×10-3的比例两组患儿的差异均无统计学意义(P>0.05)。 随访期间,TEL/AML1融 合基因阳性患儿的复发率为14.14% (14/99),无特异性融合基因组为20.97% (69/329),差异无统计学意义(χ2=2.27, P=0.132);无特异性融合基因组病死率(16.72%)高于TEL/AML1融合基因阳性组(8.08%),差异有统计学意义(χ2=4.52, P=0.033)。TEL/AML1融合基因阳性组与无特异性融合基因组的5年总生存率(OS)分别为(86.1±4.9) %和(79.0±2.8) %, 5年无复发生存率(RFS)分别为(80.7±5.1)%和(72.0±3.1)%, 5年无事件生存率(EFS)分别为(78.9±5.1)%和 (69.6±3.1)%,差异均无统计学意义(P>0.05)。COX模型分析显示第12周MRD水平是影响OS、RFS、EFS的独立预后 因素(P均<0.001)。 结论 接受CCLG-ALL-2008方案治疗的ALL患儿,TEL/AML1融合基因阳性是预后良好的指标,第 12周MRD≥1.0×10-3应推荐更强烈的治疗方案。

Abstract: Objective To evaluate the predictive role of TEL/AML1 fusion gene in protocol CCLG-ALL-2008 and to identify relevant factors influencing the outcome of ALL with TEL/AML1 fusion gene. Methods Ninety-nine patients with ALL harboring TEL/AML1 fusion gene (positive) and 329 cases without any specific fusion genes (negative) at diagnosis of B-lineage ALL from June 2008 to December 2014 were enrolled and their clinical and biological features were analyzed. Following-up ended in October 2015, the survival status was calculated by K-M curve and prognostic factors were analyzed by COX model. Results There were no differences between the two groups in age, white blood cell at the diagnostic stage, and treatment responses at 4 time points, namely, prednisone good response on day 8, M3 status of BM on D15, and the minimal residual disease (MRD) more than 1.0×10-3 on day 33 and 12th week. During the follow-up period, the relapse rate was lower in the positive group than that in the negative group (14/99 vs 69/329), the mortality rate of the negative group was twice of that in the positive group (55/329 vs 8/99). The five-year overall survival (OS) rate, relapse-free survival (RFS) rate and event-free survival (EFS) rate of the positive group were (86.1 ± 4.9)%, (80.7 ± 5.1)% and (78.9 ± 5.1)%, respectively, and (79 ±2.8)%, (72± 3.1)%, and (69.6+ 3.1)% for the negative group as well. COX regression analysis indicated that relapse and MRD level at the 12th week were independent prognostic factors on OS, RFS, and EFS (P<0.05) for the two groups. Conclusions TEL/AML1 fusion gene could be regarded as a relatively good indicator of risks in ALL children treated by CCLG-ALL-2008 protocol. ALL patients with TEL/AML1 are recommended to receive more intensive therapy including hematopoietic stem cell transplantation when the patients were high level of MRD on the 12th week after treatment.