Objective: To evaluate the efficacy and safety of rituximab combined with the modified NHL-BFM-90 protocol in childhood and adolescence with Burkitt's lymphoma (BL). Methods: A retrospective analysis of 67 untreated childhood and adolescence patients with BL was made. All patients were treated with the modified NHL-BFM-90 protocol with or without rituximab. Results: The 64 patients (95.52%) achieved complete remission (CR), 3 patients (4.48%) partial remission (PR), and the overall response rate (CR+PR) was 100%. 67 patients were followed up for a median of 44 (3-89) months. The 3 and 5-year overall survival (OS) were 92.54% and 88.98%, respectively. The 3 and 5-year progression-free survival (PFS) were all 90.34%. The 5-year OS were 100%,91.7% and 80.0% in low risk, moderate risk and high risk group, respectively, and the difference was statistically significant (P=0.048). Of the 67 patients, 55 patients (82.09%) were treated with rituximab plus chemotherapy. Compared with the 5-year OS and PFS of 74.3% and 78.6% in the chemotherapy group, the 5-year OS and PFS in the rituximab plus chemotherapy group were 95.2% and 95.5%, respectively, and the difference was statistically significant (P value was 0.021, and 0.036, respectively). Major toxicity was myelosuppression and mucositis. No treatment related death was found. Conclusions: Rituximab combined with the modified NHL-BFM-90 protocol was highly effective for children and adolescents with BL, and significantly improved long-term survival.
目的： 评估利妥昔单抗联合改良NHL-BFM-90方案对儿童和青少年伯基特淋巴瘤（BL）的远期疗效。 方法： 回顾性分析病理确诊的67例接受改良NHL-BFM-90方案±利妥昔单抗治疗的初治儿童和青少年BL患者的临床特征与疗效。 结果： 64例BL患者（95.52%）获完全缓解（CR），3例（4.48%）获部分缓解（PR），总有效率（CR+PR）为100%。中位随访44（3~89）个月，3年和5年总生存（OS）率分别为92.54%和88.98%，3年和5年无进展生存（PFS）率均为90.34%。低危、中危和高危组患者的5年OS率分别为100%、91.7%和80.0%，差异有统计学意义（P=0.048）。55例（82.09%）联合利妥昔单抗治疗，与单纯化疗组的5年OS和PFS分别为74.3%和78.6%相比，利妥昔单抗联合化疗组BL患者的5年OS和PFS分别为95.2%和95.5%，显示出明显生存优势（P值分别为0.021和0.036）。主要不良反应为骨髓抑制和黏膜炎，无治疗相关死亡。 结论： 利妥昔单抗联合改良NHL-BFM-90方案对儿童和青少年BL疗效显著，明显改善患者的长期生存率。.
Keywords: Adolescents; Burkitt′s lymphoma; Long-term survival; Rituximab.