利妥昔单抗联合改良NHL-BFM-90方案对儿童及青少年伯基特淋巴瘤的远期疗效

Long-term clinical outcome of children and adolescents with Burkitt′s lymphoma treated with rituximab combined with modified NHL-BFM-90 regimen

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。

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.