Min Gi-June, Kim Ka Young, Kim Tong Yoon, Jeon Young-Woo, Kim Byung-Su, Yahng Seung-Ah, Eom Ki-Seong, Cho Seok-Goo
Department of Hematology, Seoul St. Mary's Hospital, College of Medicine, The Catholic University of Korea, Seoul, Republic of Korea.
Department of Hematology, Yeouido St. Mary's Hospital, College of Medicine, The Catholic University of Korea, Seoul, Republic of Korea.
Front Oncol. 2025 Jul 9;15:1614506. doi: 10.3389/fonc.2025.1614506. eCollection 2025.
Burkitt lymphoma (BL), a rare, aggressive -driven B-cell non-Hodgkin lymphoma (NHL), has endemic, sporadic, and immunodeficiency-associated variants. In Asia, BL accounts for 1-2% of lymphomas, with limited data available on adult outcomes. Although potentially curable, BL is associated with poor outcomes with low-intensity chemotherapy owing to rapid proliferation and chemoresistance. Therefore, high-intensity regimens including R-hyperCVAD/MC (Course A of rituximab, cyclophosphamide, doxorubicin, vincristine, and dexamethasone; Course B of rituximab, methotrexate, and cytarabine) have been commonly used; however, no optimal strategy has been established.
This retrospective study included 69 adult patients with BL (age >15 years) diagnosed between 2009 and 2023 using the WHO criteria. Most of the patients were administered R-hyperCVAD/MC, while rituximab plus cyclophosphamide, doxorubicin, vincristine, and prednisone (R-CHOP) was administered to older patients or those with poor-performance-status to mitigate toxicity.
The median age of the patients was 55 years; 39.1% of the patients had Eastern Cooperative Oncology Group Performance Status (ECOG-PS) of 2-4, 62.3% had >1 extranodal site, 71.0% had stage IV, and 13.0% had central nervous system involvement. Furthermore, 13 (18.8%) patients were reclassified as BL after immunoglobulin heavy-chain (IGH)/ detection. Overall, 52 patients were administered R-hyperCVAD/MC exclusively, 5 switched from R-CHOP, and 4 patients were primarily treated with R-CHOP owing to intolerance. At a median follow-up of 66.9 months, 5-year overall survival (OS) and event-free survival (EFS) were 69.5 and 65.2%, respectively and higher early mortality was observed in older patients (median survival: 3.9 months). Poor OS was associated with B-symptoms (hazard ratio [HR] 3.89, = 0.003) and age ≥ 60 years (HR 2.54, = 0.034); while poor EFS was associated with ECOG-PS 2-4 (HR 2.72, = 0.024).
Our study revealed that R-hyperCVAD/MC was effective but associated with high early mortality in older patients. Risk-adapted regimens and prognostic factors including age, B-symptoms, and ECOG-PS are crucial for optimizing treatment.
伯基特淋巴瘤（BL）是一种罕见的、由侵袭性驱动的B细胞非霍奇金淋巴瘤（NHL），有地方性、散发性和免疫缺陷相关型。在亚洲，BL占淋巴瘤的1%-2%，关于成人患者预后的数据有限。尽管BL有潜在治愈可能，但由于其快速增殖和化疗耐药性，低强度化疗的预后较差。因此，包括R-hyperCVAD/MC（利妥昔单抗、环磷酰胺、阿霉素、长春新碱和地塞米松疗程A；利妥昔单抗、甲氨蝶呤和阿糖胞苷疗程B）在内的高强度方案已被普遍使用；然而，尚未确立最佳策略。
本回顾性研究纳入了69例2009年至2023年间根据世界卫生组织标准确诊的成年BL患者（年龄>15岁）。大多数患者接受了R-hyperCVAD/MC治疗，而利妥昔单抗联合环磷酰胺、阿霉素、长春新碱和泼尼松（R-CHOP）则用于老年患者或身体状况较差的患者以减轻毒性。
患者的中位年龄为55岁；39.1%的患者东部肿瘤协作组体能状态（ECOG-PS）为2-4，62.3%的患者有>1个结外部位，71.0%的患者为IV期，13.0%的患者有中枢神经系统受累。此外，13例（18.8%）患者在免疫球蛋白重链（IGH）检测后被重新分类为BL。总体而言，52例患者仅接受R-hyperCVAD/MC治疗，5例从R-CHOP转换而来，4例患者因不耐受主要接受R-CHOP治疗。中位随访66.9个月时，5年总生存率（OS）和无事件生存率（EFS）分别为69.5%和65.2%，老年患者早期死亡率更高（中位生存期：3.9个月）。OS差与B症状（风险比[HR]3.89；P = 0.003）和年龄≥60岁（HR 2.54；P = 0.034）相关；而EFS差与ECOG-PS 2-4（HR 2.72；P = 0.024）相关。
我们的研究表明，R-hyperCVAD/MC有效，但老年患者早期死亡率高。根据风险调整方案以及包括年龄、B症状和ECOG-PS在内的预后因素对于优化治疗至关重要。
