中文摘要
伯基特淋巴瘤(BL)是一种罕见且侵袭性强的B细胞淋巴瘤,特点是肿瘤增殖迅速,且常累及结外部位。儿童和青少年若能及时诊断并开始高强度化疗,治愈率可超过90%;而成人患者结局相对较差,因为合并症和年龄增长可能降低治疗耐受性。近年来,研究者为BL开发了中等强度方案。这些方案对各年龄段患者均非常有效,且治疗相关毒性显著低于传统高剂量化疗。近期一项随机研究显示,减低强度DA-EPOCH-R方案与剂量密集型R-CODOX-M/R-IVAC相比,疗效相当,但副作用显著更少。无论采用何种化疗方案,基线中枢神经系统(CNS)受累均预示结局显著较差;如何为这类患者制定最佳方案仍是BL治疗未满足的需求。前线治疗后复发或难治患者生存时间很短,因为目前可用的挽救治疗大多无效。针对这一问题,靶向CD19的CAR-T 细胞和双特异性抗体等新型药物正在BL中开发。希望新药研发取得进展,并进一步阐明BL生物学特征、遗传和表观遗传脆弱点,从而改善未来患者结局。
展开英文摘要原文
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.
论文信息
- 作者
- Lap CJ、Dunleavy K
- 单位
- Lombardi Comprehensive Cancer Center, Georgetown University Hospital, Washington, DC 20007, USA.United States
- 文献类型
- 综述
- 期刊
- Cancers2025 Oct 18