Koumasopoulos Evangelos, Spiteri Evangelia-Anna, Vakrakou Aigli G, Toulas Panagiotis, Velonakis Georgios, Stranjalis George, Stefanis Leonidas, Evangelopoulos Maria-Eleptheria, Anagnostouli Maria
Multiple Sclerosis and Demyelinating Diseases Unit, Center of Expertise for Rare Demyelinating and Autoimmune Diseases of CNS, First Department of Neurology, School of Medicine, National and Kapodistrian University of Athens, NKUA, Aeginition University Hospital, Athens, Greece.
Department of Pathology, Evaggelismos Hospital, Athens, Greece.
SAGE Open Med Case Rep. 2025 Jan 30;13:2050313X251316764. doi: 10.1177/2050313X251316764. eCollection 2025.
This is a case presentation of a primary central nervous system B-cell lymphoma in a 69-year-old woman with chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids and chronic immunosuppressive treatment. The patient had been diagnosed as having probable chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids, according to international criteria. Afterward, regular clinical and imaging examinations and blood tests were performed. The patient presented with primary central nervous system B-cell lymphoma 3 years after the initial diagnosis of chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids. Brain tissue histology was indicative of diffuse giant B cells, Epstein-Barr virus positive, and non-Hodgkin lymphoma. The nature of chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids is obscure, driving the formulation of many hypotheses about its causes. In our opinion, the presented case supports the putative neoplastic nature of the disease, at least in the long term, and/or along with Epstein-Barr virus involvement, which is known that have been related to other immune-mediated diseases such as multiple sclerosis and malignancies, especially with specific human leukocyte antigen haplotypes. Further research is needed and close monitoring of such patients is strongly recommended.
这是一例69岁女性原发性中枢神经系统B细胞淋巴瘤的病例报告，该患者患有对类固醇和慢性免疫抑制治疗有反应的伴有脑桥血管周围强化的慢性淋巴细胞性炎症。根据国际标准，该患者被诊断为可能患有对类固醇有反应的伴有脑桥血管周围强化的慢性淋巴细胞性炎症。此后，进行了定期的临床、影像学检查和血液检查。在最初诊断为对类固醇有反应的伴有脑桥血管周围强化的慢性淋巴细胞性炎症3年后，该患者出现了原发性中枢神经系统B细胞淋巴瘤。脑组织组织学显示为弥漫性大B细胞、爱泼斯坦-巴尔病毒阳性以及非霍奇金淋巴瘤。对类固醇有反应的伴有脑桥血管周围强化的慢性淋巴细胞性炎症的性质尚不清楚，引发了许多关于其病因的假说。我们认为，本病例支持该疾病至少在长期存在假定的肿瘤性质，和/或与爱泼斯坦-巴尔病毒感染有关，已知该病毒与其他免疫介导的疾病如多发性硬化症和恶性肿瘤有关，特别是与特定的人类白细胞抗原单倍型有关。需要进一步研究，并强烈建议对这类患者进行密切监测。
