改良LMB 89 C组方案治疗儿童高危伯基特淋巴瘤172例临床分析

OBJECTIVE: 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 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, 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 (χ(2)=12.939, P=0.001) and Group C2 (χ(2)=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 (χ(2)=5.972, P=0.015). Multivariable Cox regression analysis showed that Stage Ⅳ (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]. CONCLUSION: The modified LMB89 Group C regimen has ideal effect for the children with high-risk Burkitt lymphoma.