摘要目的 比较E2A-PBXl+儿童急性淋巴细胞白血病(ALL)经北京儿童医院2003方案(BCH-03组)及中国儿童白血病协作组2008方案组(CCLG-08组)治疗的效果.方法 回顾性分析2003年1月至2011年1月初治的59例E2A-PBXl+患儿的临床资料,其中BCH-03组37例,CCLG-08组22例.分析两组患儿的初诊临床特征、早期治疗反应、复发时间、无复发生存(RFS)、无事件生存(EFS)等.结果 两组患儿在性别、年龄、初诊外周血白细胞计数、中枢神经系统累及、免疫表型、早期泼尼松治疗反应及诱导缓解率等方面差异均无统计学意义(P值均＞0.05).在诱导缓解治疗阶段,BCH-03组采用60 mg/m2泼尼松,CCLG-08组采用6 mg/m2地塞米松,其他药物的剂量及治疗方式均一致.在诱导缓解治疗结束时,BCH-03组患儿的微小残留病转阴的比例显著高于CCLG-08组(84.2％ vs.47.1％,P=0.018).两组诱导缓解治疗期间感染发生率差异无统计学意义(P=0.135).BCH-03组的EFS显著优于CCLG-08组(94.5％ vs.71.5％,P=0.010),RFS可能优于CCLG-08组(94.5％ vs.78.6％,P=0.059).结论 BCH-03方案比CCLG-08方案对E2A-PBXl+儿童ALL可能具有更好的治疗效果,60 mg/m2泼尼松可能更适合此亚型白血病的诱导缓解治疗.

abstractsObjective To evaluate the efficacy of BCH-03 and CCLG-08 protocols in treating E2APBX1 pediatric acute lymphoblastic leukemia (ALL).Method From January 2003 to January 2011,59 ALL patients identified as E2A-PBX1 were analyzed in a retrospective study.There were 37 and 22 patients treated with Protocol BCH-03 and CCLG-08,respectively.The clinical characteristics at diagnosis,response to early treatment,the time of relapse,relapse-free survival (RFS) and event-free survival (EFS) in the two groups were analyzed.Result There were no significant differences in gender,age,initial white blood cell count,the central nervous system involvement,immunophenotype,prednisone response,the rate of complete remission,and the time of relapse between the two groups (P ＞ 0.05).The only difference in induction therapy of the two protocols existed in the glucocorticoids used,that is,BCH-03 used 60 mg/m2 prednisolone and CCLG-08 used 6 mg/m2 dexamethasone.The doses of vincristine,daunorubicin and Lasparaginase were the same in the two groups.At the end of induction therapy,the MRD negativity rate in BCH-03 group was significantly higher than that in CCLG-08 group (84.2％ vs.47.1％,P =0.018).The incidences of severe infection of the two groups during induction of remission were similar (P =0.135).The EFS of BCH-03 group was significantly superior to that of CCLG-08 group (94.5％ vs.71.5％,P =0.010),and the RFS of BCH-03 group tended to be better than that of CCLG-08 group (94.5％ vs.78.6％,P =0.059).Conclusion Compared to Protocol CCLG-08,Protocol BCH-03 was more effective for pediatric E2A-PBX1 ALL,and 60 mg/m2 prednisolone was more suitable for the induction therapy of this subtype of pediatric ALL.