Evens Andrew M, Gordon Leo I
Division of Hematology/Oncology, Department of Medicine, Northwestern University Feinberg School of Medicine Medical School and the Robert H. Lurie Comprehensive Cancer Center of Northwestern University, 676 N. St. Clair, Suite 850, Chicago, IL 60611, USA.
Curr Treat Options Oncol. 2002 Aug;3(4):291-305. doi: 10.1007/s11864-002-0029-9.
Burkitt's and Burkitt-like lymphoma (BL/BLL) are aggressive B-cell malignancies with a high proliferative rate that may be fatal within months if not treated promptly. Furthermore, treatment of BL/BLL requires comprehensive supportive care to avoid disease-related complications such as acute renal failure secondary to tumor lysis syndrome. Improvements in our understanding of the biology of BL and BLL have led to more effective therapeutic protocols. Clinical trials have demonstrated that short duration, multi-agent, dose-intensive chemotherapy regimens combined with aggressive central nervous system therapy results in long-term survival rates in children and young adults near 70% to 80%, whereas long-term disease-free survival rates in older adults remains suboptimal at 15% to 25%. Outcomes in HIV-associated BL/BLL are improved because of more effective chemotherapy regimens and enhanced HIV care. Autologous bone marrow transplantation has proven feasible in many patient populations with BL/BLL and may lead to cure in selected patients. Improved therapeutic strategies are warranted, such as integrating agents such as monoclonal antibodies to combination dose-intensive chemotherapy. Moreover, further study into the molecular biology of BL/BLL with attention to the role of c-myc dysregulation is needed to help predict prognostic factors and for the development of molecular targeted therapies. Clinical trials remain critical to determine the most effective treatment regimens that will continue to improve cure rates in this aggressive but treatable disease.
伯基特淋巴瘤和伯基特样淋巴瘤(BL/BLL)是侵袭性B细胞恶性肿瘤,增殖率高,若不及时治疗,可能在数月内致命。此外,BL/BLL的治疗需要全面的支持性护理,以避免疾病相关并发症,如肿瘤溶解综合征继发的急性肾衰竭。我们对BL和BLL生物学认识的提高已带来更有效的治疗方案。临床试验表明,短期、多药、剂量密集化疗方案联合积极的中枢神经系统治疗,可使儿童和年轻成人的长期生存率接近70%至80%,而老年成人的长期无病生存率仍不理想,为15%至25%。由于更有效的化疗方案和强化的HIV护理,HIV相关BL/BLL的治疗结果有所改善。自体骨髓移植已在许多BL/BLL患者群体中证明可行,可能使部分患者治愈。有必要改进治疗策略,如将单克隆抗体等药物与剂量密集联合化疗相结合。此外,需要进一步研究BL/BLL的分子生物学,关注c-myc失调的作用,以帮助预测预后因素并开发分子靶向疗法。临床试验对于确定最有效的治疗方案仍然至关重要,这些方案将继续提高这种侵袭性但可治疗疾病的治愈率。