Primary central nervous system lymphoma in neuropsychiatric systemic lupus erythematosus: case-based review.
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.
['Ichikawa|Takanori|T|http://orcid.org/0000-0001-6570-5878', 'Shimojima|Yasuhiro|Y|http://orcid.org/0000-0001-7100-1121', 'Kishida|Dai|D|http://orcid.org/0000-0001-8309-7984', 'Kaneko|Tomoki|T|http://orcid.org/0000-0002-2905-4227', 'Sekijima|Yoshiki|Y|http://orcid.org/0000-0002-8132-2743']
D000328:Adult D061067:Antibodies, Monoclonal, Humanized D000074322:Antineoplastic Agents, Immunological D001921:Brain D005260:Female D004854:Herpesvirus 4, Human D006801:Humans D007165:Immunosuppression Therapy D007166:Immunosuppressive Agents D020945:Lupus Vasculitis, Central Nervous System D016403:Lymphoma, Large B-Cell, Diffuse D000069283:Rituximab
Primary central nervous system lymphoma Neuropsychiatric systemic lupus erythematosus Systemic lupus erythematosus Immunosuppressant Epstein–Barr virus