Central nervous system B-cell lymphoma in a patient diagnosed with chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids: A case report.

Abstract

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.