Department of Medicine (Neurology and Rheumatology), Shinshu University School of Medicine, 3-1-1 Asahi, Matsumoto, 390-8621, Japan.
Department of Radiology, Shinshu University School of Medicine, 3-1-1 Asahi, Matsumoto, 390-8621, Japan.
Rheumatol Int. 2021 May;41(5):1009-1017. doi: 10.1007/s00296-020-04569-6. Epub 2020 Apr 6.
Primary central nervous system lymphoma (PCNSL) sometimes occurs in immune-compromised hosts or patients with autoimmune diseases. Some cohort studies have previously reported an increased risk of non-Hodgkin's lymphoma in systemic lupus erythematosus (SLE), while some cases of PCNSL in patients with SLE were reported. We present the case of PCNSL which developed in a patient with the active phase of neuropsychiatric SLE (NPSLE). Furthermore, we reviewed published English articles to confirm the characteristics of PCNSL related to SLE. To our knowledge, this is the first report of PCNSL occurring in NPSLE. Histology demonstrated B-cell lymphoma with a positive Epstein-Barr virus-encoded RNA. This patient recovered following surgical resection of the lymphoma, whole brain radiation therapy, intravenous infusion of rituximab (RTX), and administration of belimumab after RTX. Given the series of reviews, our report suggests that the persistence of damage in the central nervous system (CNS) and long-term exposure to immunosuppressants may impact oncogenic immune responses within the CNS, leading to PCNSL development.
原发性中枢神经系统淋巴瘤（PCNSL）有时发生于免疫功能低下的宿主或自身免疫性疾病患者中。一些队列研究先前报道系统性红斑狼疮（SLE）患者中非霍奇金淋巴瘤的风险增加，同时也有 SLE 患者发生 PCNSL 的病例报告。我们报告了一例活动期神经精神性 SLE（NPSLE）患者发生的 PCNSL。此外，我们回顾了已发表的英文文献，以确认与 SLE 相关的 PCNSL 的特征。据我们所知，这是首例发生于 NPSLE 的 PCNSL 病例。组织学显示为 B 细胞淋巴瘤，EB 病毒编码的 RNA 呈阳性。该患者在接受淋巴瘤切除术、全脑放疗、静脉输注利妥昔单抗（RTX）以及 RTX 后使用贝利尤单抗治疗后康复。基于一系列文献复习，我们的报告表明中枢神经系统（CNS）持续受损和长期暴露于免疫抑制剂可能会影响 CNS 内的致癌免疫反应，从而导致 PCNSL 的发生。
