全身性弥漫大B细胞淋巴瘤（DLBCL）继发于中枢神经系统（CNS）时，预后非常差。我们报告了一名女性患者，她曾经有两次在脑内发生的CNS-only复发，是全身非生发中心性大B细胞淋巴瘤（NGC-DLBCL）的表现。她在初次诊断时接受了利妥昔单抗，环磷酰胺，阿霉素，长春新碱和泼尼松（R-CHOP）以及脑脊液内注射甲氨蝶呤（MTX）的诱导治疗，随后在高剂量卡姆司汀，依托泊苷，阿糖胞苷和甲氨蝶呤（BEAM）化疗后进行自体干细胞移植（ASCT）。她在ASCT后1.5年首次出现仅限于CNS的复发，并接受了全脑放疗（WBRT）。她在WBRT后2年出现了第二次脑内CNS-only复发。对于她的第二次CNS-only复发，采用了以脑为中心的salvage化学免疫治疗，包括利妥昔单抗，高剂量甲氨蝶呤（HD-MTX），高剂量阿糖胞苷（HiDAC）和伊布替尼。她在高剂量卡姆司汀（BCNU）和替曲奈派化疗后进行了第二次ASCT的巩固治疗。鉴于她复发中枢神经系统的高风险，她开始接受维持治疗伊布替尼。到目前为止，她已经持续缓解了3年。根据我们的经验，具有这种反应的多次复发的继发性CNS淋巴瘤（SCNSL）非常罕见。我们建议采用一种以中枢神经系统为中心的治疗方案，可以潜在地挽救那些以前没有接受足够的中枢神经系统定向治疗的SCNSL患者，但是我们承认需要进行额外的研究来验证我们的发现。© 2023 Fournier等人。

Secondary central nervous system involvement by systemic diffuse large B-cell lymphoma (DLBCL) carries a very poor prognosis. We present a female patient who had two episodes of intracerebral central nervous system (CNS)-only relapse of systemic non-germinal center diffuse large B-cell lymphoma (NGC-DLBCL). Her treatment at initial diagnosis consisted of induction with rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone (R-CHOP) and intrathecal (IT) - methotrexate (MTX) followed by consolidation with autologous stem cell transplant (ASCT) after high-dose carmustine, etoposide, cytarabine, and melphalan (BEAM) chemotherapy. She had the first CNS-only relapse 1.5 years post-ASCT and received whole brain radiation therapy (WBRT). She developed the second intracerebral CNS-only relapse 2 years post-WBRT. A CNS-centric therapeutic approach with salvage chemoimmunotherapy incorporating rituximab, high-dose methotrexate (HD-MTX), high-dose cytarabine (HiDAC), and ibrutinib was utilized for her second CNS-only relapse. She underwent consolidation with a second ASCT following high-dose carmustine (BCNU) and thiotepa chemotherapy. Given her high risk of CNS recurrence, she was started on maintenance ibrutinib. To date, she has remained in complete remission for 3 years. In our experience, multiply relapsed secondary CNS lymphoma (SCNSL) with this response is very rare. We suggest one CNS-centric therapeutic approach that can potentially salvage patients with SCNSL who have not had prior exposure to adequate CNS-directed therapies but acknowledge that additional research is necessary to validate our findings.© 2023 Fournier et al.