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

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...

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Main Authors: Evangelos Koumasopoulos, Evangelia-Anna Spiteri, Aigli G Vakrakou, Panagiotis Toulas, Georgios Velonakis, George Stranjalis, Leonidas Stefanis, Maria-Eleptheria Evangelopoulos, Maria Anagnostouli
Format: Article
Language:English
Published: SAGE Publishing 2025-01-01
Series:SAGE Open Medical Case Reports
Online Access:https://doi.org/10.1177/2050313X251316764
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author Evangelos Koumasopoulos
Evangelia-Anna Spiteri
Aigli G Vakrakou
Panagiotis Toulas
Georgios Velonakis
George Stranjalis
Leonidas Stefanis
Maria-Eleptheria Evangelopoulos
Maria Anagnostouli
author_facet Evangelos Koumasopoulos
Evangelia-Anna Spiteri
Aigli G Vakrakou
Panagiotis Toulas
Georgios Velonakis
George Stranjalis
Leonidas Stefanis
Maria-Eleptheria Evangelopoulos
Maria Anagnostouli
author_sort Evangelos Koumasopoulos
collection DOAJ
description 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.
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spelling doaj-art-98968906043440ceb561b4ffe1f094472025-01-30T16:03:57ZengSAGE PublishingSAGE Open Medical Case Reports2050-313X2025-01-011310.1177/2050313X251316764Central nervous system B-cell lymphoma in a patient diagnosed with chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids: A case reportEvangelos Koumasopoulos0Evangelia-Anna Spiteri1Aigli G Vakrakou2Panagiotis Toulas3Georgios Velonakis4George Stranjalis5Leonidas Stefanis6Maria-Eleptheria Evangelopoulos7Maria Anagnostouli8Multiple 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, GreeceDepartment of Pathology, Evaggelismos Hospital, Athens, GreeceMultiple 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, GreeceResearch Unit of Radiology, Second Department of Radiology, Aeginition University Hospital, National and Kapodistrian University of Athens, Athens, GreeceResearch Unit of Radiology, Second Department of Radiology, Aeginition University Hospital, National and Kapodistrian University of Athens, Athens, GreeceDepartment of Neurosurgery, National and Kapodistrian University of Athens, Athens, GreeceFirst Department of Neurology. School of Medicine, National and Kapodistrian University of Athens, NKUA, Aeginition University Hospital, Athens, GreeceMultiple 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, GreeceMultiple 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, GreeceThis 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.https://doi.org/10.1177/2050313X251316764
spellingShingle Evangelos Koumasopoulos
Evangelia-Anna Spiteri
Aigli G Vakrakou
Panagiotis Toulas
Georgios Velonakis
George Stranjalis
Leonidas Stefanis
Maria-Eleptheria Evangelopoulos
Maria Anagnostouli
Central nervous system B-cell lymphoma in a patient diagnosed with chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids: A case report
SAGE Open Medical Case Reports
title Central nervous system B-cell lymphoma in a patient diagnosed with chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids: A case report
title_full Central nervous system B-cell lymphoma in a patient diagnosed with chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids: A case report
title_fullStr Central nervous system B-cell lymphoma in a patient diagnosed with chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids: A case report
title_full_unstemmed Central nervous system B-cell lymphoma in a patient diagnosed with chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids: A case report
title_short Central nervous system B-cell lymphoma in a patient diagnosed with chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids: A case report
title_sort central nervous system b cell lymphoma in a patient diagnosed with chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids a case report
url https://doi.org/10.1177/2050313X251316764
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