Burkitt lymphoma (BL) is a rare, aggressive B-cell lymphoma that is characterized by rapid tumor proliferation and frequent extra-nodal involvement. While prompt diagnosis and initiation of highly intensive chemotherapy results in cure rates over 90% in children and adolescents, outcomes in adults are more modest, as comorbidities and advancing age may compromise treatment tolerability. In recent years, intermediate-intensity regimens have been developed for BL. These are highly effective in patients of all ages and associated with significantly less treatment-related toxicity compared to traditional high-dose chemotherapy. This was demonstrated in a recent randomized study of dose-intensive R-CODOX-M/R-IVAC compared to the reduced-intensity DA-EPOCH-R regimen, which was associated with equivalent outcomes but with significantly fewer side effects. Regardless of the chemotherapy platform, CNS involvement at baseline predicts a significantly inferior outcome, and the development of an optimal approach for these patients is an area of unmet need in BL therapeutics. Patients with relapsed or refractory disease following frontline therapy have very short survival times, as currently available salvage options are largely ineffective. In this regard, novel agents such as anti-CD19 CAR-T cells and bi-specific antibodies are under development in BL. It is hoped that progress in novel drug development, alongside improved understanding of BL biology, to further elucidate its genetic and epigenetic vulnerabilities, will lead to improved outcomes for patients in the future.
伯基特淋巴瘤（BL）是一种罕见且侵袭性强的B细胞淋巴瘤，其特点是肿瘤增殖迅速并常伴有结外侵犯。尽管及时诊断并启动高强度化疗可使儿童和青少年患者的治愈率超过90%，但成人患者的治疗效果相对有限，因为合并症和年龄增长可能影响治疗耐受性。近年来，针对BL的中等强度治疗方案已得到发展。这些方案对所有年龄段患者均高度有效，且与传统高剂量化疗相比，治疗相关毒性显著降低。最近一项随机研究对此进行了验证，该研究比较了剂量密集型R-CODOX-M/R-IVAC方案与减量强度DA-EPOCH-R方案，结果显示两者疗效相当，但后者副作用显著减少。无论采用何种化疗方案，基线存在中枢神经系统侵犯均提示预后显著较差，针对这类患者制定最佳治疗方案仍是BL治疗领域尚未满足的需求。一线治疗后复发或难治的患者生存期极短，目前可用的挽救治疗方案大多效果有限。为此，针对BL的新型药物如抗CD19 CAR-T细胞和双特异性抗体正在研发中。未来，随着新药研发的进展以及对BL生物学特性的深入理解，进一步阐明其遗传和表观遗传脆弱性，有望改善患者的临床结局。
Novel Molecular Insights and Evolution of Less Toxic Therapeutic Strategies in Burkitt Lymphoma
肿瘤(癌症)患者之家