摘要目的 分析改良LMB 89 C组方案治疗高危儿童伯基特淋巴瘤的疗效.方法 对2007年1月至2017年4月接受改良LMB 89 C组方案治疗的172例初治高危伯基特淋巴瘤患儿进行回顾性分析.结果 全部172例患儿中位发病年龄6(1～14)岁,男144例(83.7％),女28例(16.3％),St.Jude分期Ⅱ、Ⅲ、Ⅳ期分别为2例(1.2％)、54例(31.4％)、116例(67.4％),46例(26.7％)为白血病期,52例(30.2％)存在中枢神经系统(CNS)侵犯.危险度分组:C1组(CNS1,无睾丸/卵巢侵犯)65例,C2组[CNS2和(或)睾丸/卵巢侵犯]55例,C3组(CNS3)52例.145例联合应用利妥昔单抗.10例未缓解并进展至死亡,5例复发,治疗相关死亡率为2.9％.中位随访时间36.0(0.5～119.0)个月,全组3年总生存(OS)率为(88.9±2.4)％、3年无事件生存(EFS)率为(87.9±2.6)％.C1、C2、C3组3年EFS率分别为(96.9±2.1)％、(90.9±3.9)％、(73.4±6.5)％,C3组低于C1组(χ2=12.939,P=0.001)和C2组(χ2=6.302,P=0.036).C3组中利妥昔单抗联合化疗、单纯化疗患儿的3年EFS率分别为(79.3±6.8)％、(44.4±16.6)％(χ2=5.972,P=0.015).多因素分析结果显示,临床分期Ⅳ期(包括白血病期)、中期评估有瘤灶为预后不良的危险因素[HR=4.241(95％CI 1.163～27.332),P=0.026;HR=32.184(95％CI 11.441～99.996),P<0.001].结论 改良LMB 89 C组方案对于高危儿童伯基特淋巴瘤具有较理想的疗效.

abstractsObjective To analyze the therapeutic effect of a modified LMB89 Group C regimen in the treatment of pediatric high-risk Burkitt lymphoma. Methods The clinical data of 172 children with newly diagnosed high-risk Burkitt lymphoma from January 2007 to April 2017 were retrospectively analyzed. All the cases were treated with the modified LMB89 Group C regimen. Results The median age of the patients was 6(1-14)years. The sex ratio was 5.1:1, 144 boys(83.7％)and 28 girls(16.3％). According to St. Jude staging classification, 2 patients(1.2％)were in stageⅡ, 54(31.4％)in stage Ⅲ and 116 (67.4％) in stage Ⅳ. Of them, 46 patients (26.7％) had mature B cell acute lymphoblastic leukemia(B-ALL), and 52 patients had central nervous system(CNS)involvement. According to risk group, the patients can be divided into group C1(CNS1, without testicles/ovaries involvement, n=65), group C2 (CNS2, testicles/ovaries involvement, n=55) and group C3 (CNS3, n=52). A total of 145 patients received rituximab combined with chemotherapy during the treatment, 10 patients suffered from progressive disease and died, and 5 patients relapsed. Treatment-related mortality was 2.9％. With a median follow-up of 36.0(0.5-119.0)months, 3-year overall survival(OS)rate was(88.9±2.4)％and event free survival(EFS)rate was(87.9±2.6)％for all patients. 3-year EFS rates were(96.9±2.1)％,(90.9±3.9)％and(73.4±6.5)％for Group C1, C2 and C3 respectively, and that of Group C3 was significantly lower than that of Group C1(χ2=12.939, P=0.001)and Group C2(χ2=6.302, P=0.036). The 3-year EFS rates were(79.3±6.8)％and(44.4±16.6)％for patients in group C3 treated with chemotherapy combined with rituximab and chemotherapy alone(χ2=5.972, P=0.015). Multivariable Cox regression analysis showed that StageⅣ(including B-ALL), residual diseases in mid-term evaluation were independent unfavorable prognostic factors[HR=4.241(95％CI 1.163-27.332), P=0.026;HR=32.184(95％CI 11.441-99.996), P<0.001]. Conclusions The modified LMB89 Group C regimen has ideal effect for the children with high-risk Burkitt lymphoma.