IRCCS Azienda Ospedaliero-Universitaria di Bologna, Istituto di Ematologia "Seràgnoli," Bologna, Italy.
Dipartimento di Scienze Mediche e Chirurgiche, Università di Bologna, Bologna, Italy.
Oncologist. 2024 Jun 3;29(6):e789-e795. doi: 10.1093/oncolo/oyae017.
Intensive treatment approaches are required for adult patients with Burkitt lymphoma (BL), although an univocal standard of care still does not exist. The use of frontline autologous stem cells transplantation (ASCT) is debated.
Between 2004 and 2020, 50 patients with BL were treated with the Berlin-Frankfurt-Münster (BFM). Treatment plan consisted of 3 blocks, A (ifosfamide, vincristine, methotrexate, etoposide, and cytarabine), B (vincristine, cyclophosphamide, methotrexate, and doxorubicin), and C (vindesine, methotrexate, etoposide, and cytarabine), each repeated twice, every 28 days. Rituximab was given at day 1 each block. Intrathecal prophylaxis was given once per each block. ASCT was scheduled at the end of the 6 blocks after conditioning.
Median age at onset was 38 years (range 16-72); stages III-IV disease was observed in 82% of cases; bulky disease occurred in 44% of the patients, with B-symptoms in 38%. Stem cell harvest was performed in 72% of patients, who all received a subsequent ASCT. The full 6 blocks treatment was completed in 70% of the patients. The overall response rate was 74%, with a complete response rate of 60%. Ten-year overall survival and progression-free survival were 83.7% and 76.0%, respectively, without reaching the median. Ten-year disease-free survival was 80.3%. Grades 3-4 neutropenia, thrombocytopenia, anemia, and mucositis were seen in 96%, 60%, 32%, and 24% of patients. Infections occurred in 60% of patients.
Intensive treatment according to BFM protocol, with rituximab and ASCT, appears feasible, safe, and highly effective in adult patients with BL, as confirmed by long-term survival rates reflecting response maintenance.
尽管目前尚无统一的治疗标准，但成人伯基特淋巴瘤（BL）患者需要强化治疗方法。一线自体干细胞移植（ASCT）的应用存在争议。
2004 年至 2020 年间，50 例 BL 患者接受了柏林-法兰克福-明斯特（BFM）治疗。治疗方案包括 3 个阶段，A 阶段（异环磷酰胺、长春新碱、甲氨蝶呤、依托泊苷和阿糖胞苷）、B 阶段（长春新碱、环磷酰胺、甲氨蝶呤和多柔比星）和 C 阶段（长春花碱、甲氨蝶呤、依托泊苷和阿糖胞苷），每个阶段重复 2 次，每 28 天一次。每个阶段第一天给予利妥昔单抗。每个阶段给予一次鞘内预防。在 6 个阶段结束后进行 ASCT 预处理。
中位发病年龄为 38 岁（范围 16-72 岁）；82%的病例为 III-IV 期疾病；44%的患者有肿块，38%的患者有 B 症状。72%的患者进行了干细胞采集，均接受了随后的 ASCT。70%的患者完成了完整的 6 个阶段治疗。总体缓解率为 74%，完全缓解率为 60%。10 年总生存率和无进展生存率分别为 83.7%和 76.0%，未达到中位数。10 年无病生存率为 80.3%。96%、60%、32%和 24%的患者分别出现 3-4 级中性粒细胞减少、血小板减少、贫血和黏膜炎。60%的患者发生感染。
根据 BFM 方案，采用利妥昔单抗和 ASCT 的强化治疗，在成人 BL 患者中是可行的、安全的且高度有效的，长期生存率反映了缓解的维持。
