继发性中枢神经系统淋巴瘤（SCNSL）是一种罕见但临床上具有挑战性的情况，其历史上的疗效令人失望。SCNSL指的是在全身性疾病同时进入中枢神经系统，或在前线免疫化疗期间或之后出现中枢神经系统复发的淋巴瘤，有或无全身性淋巴瘤的表现。弥漫性大B细胞淋巴瘤（DLBCL）是最常见的类型，但在其他组织学类型（如布基特淋巴瘤和套膜细胞淋巴瘤）中观察到发病率增加。发病率、疾病进程中的时机、部位、支持中枢神经系统预防治疗的证据以及治疗路径在不同组织学类型中各不相同。目前没有随机数据来阐明最佳治疗方法，现行的建议基于回顾性和单臂研究。然而，一项以能穿越血脑屏障的药物为基础的免疫化疗方案，以硫砹泰巴联合条件化疗和自体干细胞移植为后继治疗，在国际MARIETTA研究中显示出疗效改善，成为DLBCL患者SCNSL治疗的重要进展。CD19嵌合抗原受体T细胞（CAR-T细胞）在治疗全身性侵袭性淋巴瘤患者中具有革命性意义，新兴数据还显示在SCNSL患者中具有同样的疗效且没有更高的神经毒性。本文将讨论五种临床情况并回顾支持我们建议的证据。版权所有 © 2023年美国血液学会。

Secondary central nervous system lymphoma (SCNSL) is a rare but clinically challenging scenario with historically disappointing outcomes. SCNSL refers to lymphoma that has spread into the CNS concurrently with systemic disease, or CNS relapse during or after frontline immunochemotherapy, presenting with or without systemic lymphoma. Diffuse large B-cell lymphoma (DLBCL) denotes the most common entity but an increased incidence is observed in other histologies such as Burkitt lymphoma and mantle cell lymphoma. The incidence, timing in disease course, location, evidence supporting the use of CNS prophylaxis, and treatment pathways varies by histology. No randomized data exist to delineate the best treatment approaches with current recommendations based on retrospective and single-arm studies. However, a regimen comprised of immunochemotherapy, incorporating agents that cross the blood-brain barrier, followed by thiotepa-containing conditioning and autologous stem-cell transplant outlined in the international MARIETTA study demonstrated improvement in outcomes representing a major accomplishment in the care of DLBCL patients with SCNSL. Anti-CD19 chimeric antigen receptor T-cell denotes a paradigm shift in the treatment of patients with systemic aggressive lymphomas with emerging data also demonstrating efficacy without higher neurotoxicity in those with SCNSL. In this manuscript we discuss five clinical scenarios and review the evidence supporting our recommendations.Copyright © 2023 American Society of Hematology.