Dlouhy Ivan, Viegas Diana, Coelho Inês, Ionita Alina, Carvalho Susana, Cabeçadas José, da Silva Maria Gomes
Hematology Department, Instituto Português de Oncologia de Lisboa Francisco Gentil, 1099-023 Lisboa, Portugal.
Pathology Department, Instituto Português de Oncologia de Lisboa Francisco Gentil, 1099-023 Lisboa, Portugal.
Cancers (Basel). 2025 Sep 5;17(17):2914. doi: 10.3390/cancers17172914.
: Burkitt lymphoma is a rare, aggressive B-cell neoplasm with frequent central nervous system (CNS) involvement, treated with intensive multidrug regimens associated with rituximab. The aim of this study was to assess the efficacy, safety, and feasibility of the LMB protocol in adults with BL in a real-world setting. : We included 55 patients with BL diagnosis according to the 2008 WHO classification, treated with LMB protocol associated with rituximab. Low-risk patients (no bone marrow or CNS involvement) were treated in the group B arm, while high-risk patients were placed in group C, which was further stratified by age and CNS infiltration. : Thirty-four patients (62%) were treated in group B and 21 patients (38%) were treated in group C, with a median age of 34 years (16-77). Extranodal infiltration was present in 71% patients, including 11 (20%) with CNS involvement. After a median follow up time of 7 years, the complete remission rate was 85%, and progression-free and overall survival at 3 years were 79% and 84%, respectively. Patients with CNS infiltration had an inferior survival rate (55% at 3 years). Grade 3-4 toxicities were frequent, mainly hematologic, infectious, and mucosal. Treatment required substantial supportive care, including 1604 transfusions and 4696 days of hospitalization. Patients over 60 years had poorer outcomes and higher toxicity. : The LMB protocol demonstrated high survival rates in adult BL, although at the cost of significant toxicity and considerable health care resource utilization. Outcomes remained suboptimal in patients with CNS involvement despite treatment intensification.
伯基特淋巴瘤是一种罕见的侵袭性B细胞肿瘤，常累及中枢神经系统（CNS），采用与利妥昔单抗联合的强化多药方案治疗。本研究的目的是在真实世界环境中评估LMB方案治疗成人伯基特淋巴瘤的疗效、安全性和可行性。我们纳入了55例根据2008年世界卫生组织分类诊断为伯基特淋巴瘤的患者，采用与利妥昔单抗联合的LMB方案治疗。低风险患者（无骨髓或中枢神经系统受累）在B组接受治疗，而高风险患者被纳入C组，C组再根据年龄和中枢神经系统浸润情况进一步分层。34例患者（62%）在B组接受治疗，21例患者（38%）在C组接受治疗，中位年龄为34岁（16 - 77岁）。71%的患者存在结外浸润，其中11例（20%）有中枢神经系统受累。中位随访7年后，完全缓解率为85%，3年无进展生存率和总生存率分别为79%和84%。有中枢神经系统浸润的患者生存率较低（3年时为55%）。3 - 4级毒性反应常见，主要为血液学、感染性和黏膜性。治疗需要大量的支持治疗，包括1604次输血和4696天的住院治疗。60岁以上的患者预后较差，毒性反应较高。LMB方案在成人伯基特淋巴瘤中显示出较高的生存率，尽管代价是显著的毒性反应和大量的医疗资源利用。尽管强化了治疗，但中枢神经系统受累患者的预后仍然欠佳。
