Zhang M, Jin L, Yang J, Duan Y L, Huang S, Zhou C J, Zhang Y H
Beijing Key Laboratory of Pediatric Hematology Oncology; National Discipline of Pediatrics, Ministry of Education; MOE Key Laboratory of Major Diseases in Children; Hematology Oncology Center, Beijing 100045, China.
Pathology Department, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing 100045, China.
Zhonghua Xue Ye Xue Za Zhi. 2019 Aug 14;40(8):633-638. doi: 10.3760/cma.j.issn.0253-2727.2019.08.002.
To analyze the therapeutic effect of a modified LMB89 Group C regimen in the treatment of pediatric high-risk Burkitt lymphoma. 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. 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 stage Ⅱ, 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, =65) , group C2 (CNS2, testicles/ovaries involvement, =55) and group C3 (CNS3, =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 ((2)=12.939, =0.001) and Group C2 ((2)=6.302, =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 ((2)=5.972, =0.015) . Multivariable Cox regression analysis showed that Stage Ⅳ (including B-ALL) , residual diseases in mid-term evaluation were independent unfavorable prognostic factors[=4.241 (95% 1.163-27.332) , =0.026; =32.184 (95% 11.441-99.996) , <0.001]. The modified LMB89 Group C regimen has ideal effect for the children with high-risk Burkitt lymphoma.
分析改良LMB89 C组方案治疗儿童高危伯基特淋巴瘤的疗效。回顾性分析2007年1月至2017年4月期间172例新诊断的高危伯基特淋巴瘤患儿的临床资料。所有病例均采用改良LMB89 C组方案治疗。患者的中位年龄为6（1 - 14）岁。男女比例为5.1∶1，男孩144例（83.7%），女孩28例（16.3%）。根据圣裘德分期分类，Ⅱ期2例（1.2%），Ⅲ期54例（31.4%），Ⅳ期116例（67.4%）。其中，46例（26.7%）患有成熟B细胞急性淋巴细胞白血病（B - ALL），52例有中枢神经系统（CNS）受累。根据危险组，患者可分为C1组（CNS1，无睾丸/卵巢受累，n = 65）、C2组（CNS2，睾丸/卵巢受累，n = 55）和C3组（CNS3，n = 52）。共有145例患者在治疗期间接受了利妥昔单抗联合化疗，10例患者病情进展死亡，5例复发。治疗相关死亡率为2.9%。中位随访36.0（0.5 - 119.0）个月，所有患者的3年总生存率（OS）为（88.9±2.4）%，无事件生存率（EFS）为（87.9±2.6）%。C1组、C2组和C3组的3年EFS率分别为（96.9±2.1）%、（90.9±3.9）%和（73.4±6.5）%，C3组显著低于C1组（χ² = 12.939，P = 0.001）和C2组（χ² = 6.302，P = 0.036）。C3组中接受化疗联合利妥昔单抗和单纯化疗的患者3年EFS率分别为（79.3±6.8）%和（44.4±16.6）%（χ² = 5.972，P = 0.015）。多因素Cox回归分析显示，Ⅳ期（包括B - ALL）、中期评估有残留病灶是独立的不良预后因素[HR = 4.241（95% 1.163 - 27.332）, P = 0.026；HR = 32.184（95% 11.441 - 99.99）, P < 0.001]。改良LMB89 C组方案治疗儿童高危伯基特淋巴瘤疗效理想。
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