Advanced Medical Research Center, Aichi Medical University, Aichi, Japan.
Pediatr Blood Cancer. 2014 Jul;61(7):1215-21. doi: 10.1002/pbc.24975. Epub 2014 Feb 13.
Previous Japanese studies of childhood B-cell non-Hodgkin lymphoma (B-NHL) have shown a favorable outcome, though the study size was too small to effectively assess the efficacy and safety of treatment for childhood B-NHL.
We performed a nation-wide prospective B-NHL03 study to assess the efficacy and safety of short-pulse intensive chemotherapy for children with B-NHL. They were stratified into four treatment groups according to disease stage, tumor resectability and bone marrow/CNS involvement: Group 1 with all resected stage I/II, Group 2 with non-resected stage I/II, Group 3 with stage III & CNS-negative stage IV, and Group 4 with CNS-positive stage IV & Burkitt leukemia. Treatment duration was 2 courses for Group 1, 4 courses for Group 2, and 6 courses for Groups 3 and 4, respectively. CNS irradiation was omitted in all patients.
The follow-up time ranged from 0.8 to 88 months, with a median of being 45 months. For 321 patients analyzed in this study, overall survival and event-free survival (EFS) at 4 years was 92.7% and 87.4%, respectively. The 4-year EFS according to treatment group were 94% for Group 1 (n = 17), 98% for Group 2 (n = 103), 84% for Group 3 (n = 111), and 78% for Group 4 (n = 90). There was no significant difference in outcome by histology. Therapy-related death occurred in three patients in remission.
Our nationwide large-scale study resulted in a cure rate above 90% with <1% toxic death in childhood B-NHL.
之前的日本儿童 B 细胞非霍奇金淋巴瘤（B-NHL）研究显示预后良好，尽管研究规模太小，无法有效评估儿童 B-NHL 治疗的疗效和安全性。
我们进行了一项全国性的前瞻性 B-NHL03 研究，以评估短脉冲强化化疗治疗儿童 B-NHL 的疗效和安全性。根据疾病分期、肿瘤可切除性和骨髓/中枢神经系统受累情况，将患儿分为四个治疗组：I/II 期完全切除的 1 组、I/II 期未切除的 2 组、无中枢神经系统受累的 III 期和 IV 期的 3 组，以及有中枢神经系统受累的 IV 期和伯基特白血病的 4 组。1 组治疗持续 2 个疗程，2 组治疗持续 4 个疗程，3 组和 4 组分别治疗 6 个疗程。所有患者均未接受中枢神经系统照射。
随访时间为 0.8 至 88 个月，中位数为 45 个月。本研究分析了 321 例患儿，4 年总生存率和无事件生存率（EFS）分别为 92.7%和 87.4%。根据治疗组，4 年 EFS 分别为 1 组（n=17）94%、2 组（n=103）98%、3 组（n=111）84%和 4 组（n=90）78%。组织学无显著差异。缓解期有 3 例患儿死亡。
我们的全国性大规模研究使儿童 B-NHL 的治愈率超过 90%，毒性死亡<1%。
