Abstract：
Central nervous system (CNS) lymphoma has traditionally had very poor outcomes however advances in management have resulted in dramatic improvements and long-term survival of patients. We describe the evidence for treatment strategies for these aggressive disorders. In primary CNS lymphoma there are randomized trial data to inform treatment decisions but these are lacking to guide management in secondary CNS lymphoma. Dynamic assessment of patient fitness and frailty is key throughout treatment, alongside delivery of CNS-bioavailable therapy and enrolment in clinical trials, at each stage of the disease. Intensive high-dose methotrexate-containing induction followed by consolidation with autologous stem cell transplantation with thiotepa-based conditioning is recommended for patients who are fit. Less intensive chemoimmunotherapy, novel agents (including Bruton tyrosine kinase inhibitors, cereblon targeting immunomodulatory agents, and checkpoint inhibitors in the context of clinical trials), and whole brain radiotherapy may be reserved for less fit patients or disease which is chemoresistant. Data regarding the efficacy of chimeric antigen receptor T-cells therapy is emerging, and concerns regarding greater toxicity have not been realized. Future areas of prospective studies include the identification of those at high risk of developing CNS lymphoma, management in elderly or frail patients as well as incorporating novel agents into regimens, particularly for those with chemoresistant disease.
摘要：
中枢神经系统（CNS）淋巴瘤传统上具有非常差的结果，但是管理方面的进步已导致患者的显着改善和长期生存。我们描述了这些侵袭性疾病的治疗策略的证据。在原发性中枢神经系统淋巴瘤中，有随机试验数据可告知治疗决策，但这些数据缺乏指导继发性中枢神经系统淋巴瘤的管理。患者健康和虚弱的动态评估是整个治疗过程中的关键，除了提供中枢神经系统生物可利用性治疗和参加临床试验，在疾病的每个阶段。对于健康的患者，建议进行高强度的含甲氨蝶呤的高剂量诱导，然后进行自体干细胞移植并进行基于噻替帕的预处理。不太密集的化学免疫疗法，新型药物（包括布鲁顿酪氨酸激酶抑制剂，小脑靶向免疫调节剂，和临床试验中的检查点抑制剂)，全脑放疗可能保留给不太适合的患者或化疗耐药的疾病。关于嵌合抗原受体T细胞疗法疗效的数据正在出现，和对更大毒性的担忧尚未实现。未来的前瞻性研究领域包括确定那些在发展中枢神经系统淋巴瘤的高风险，对老年或虚弱患者的管理，以及将新型药物纳入治疗方案，特别是对于那些患有化学抗性疾病的人。
