scholarly journals Chronic lymphocytic infiltration with pontine perivascular enhancement responsive to steroids (CLIPPERS) and its association with Epstein‐Barr Virus (EBV)-related lymphomatoid granulomatosis: a case report

BMC Neurology ◽  
2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Yew Li Dang ◽  
Hong Kuan Kok ◽  
Penelope A. McKelvie ◽  
Matthew Ligtermoet ◽  
Laura Maddy ◽  
...  
Keyword(s):  
Epstein Barr Virus ◽  
Brain Biopsy ◽  
Initial Response ◽  
Lymphocytic Infiltration ◽  
Lymphoproliferative Disorders ◽  
Lymphomatoid Granulomatosis ◽  
Barr Virus ◽  
Epstein Barr ◽  
Granulomatous Diseases ◽  
Inflammatory Syndrome

Abstract Background Chronic lymphocytic infiltration with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a neuro-inflammatory syndrome first described in 2010. It has a relationship with lymphoproliferative disorders that has not been fully elucidated. This case represents an unusual progression of CLIPPERS to Epstein-Barr Virus (EBV)-related lymphomatoid granulomatosis (LYG). The exact connection between CLIPPERS and LYG remains poorly understood. Case presentation We present a case of a 75-year-old man who was diagnosed with CLIPPERS with initial response to immunosuppression but later progressed to EBV-related LYG. EBV polymerase chain reaction (PCR) was detected in his cerebrospinal fluid (CSF), and repeat imaging revealed findings that were uncharacteristic for CLIPPERS; thereby prompting a brain biopsy which led to a diagnosis of EBV-related LYG. This case highlights the following learning points: 1) CLIPPERS cases are often part of a spectrum of lymphomatous disease, 2) CLIPPERS can be associated with EBV-related lymphoproliferative disorders such as LYG, and 3) EBV detection in CSF should prompt earlier consideration for brain biopsy in patients. Conclusions Our case highlights the difficulty in distinguishing CLIPPERS from other steroid-responsive conditions such as neoplastic and granulomatous diseases. Given the association of CLIPPERS with EBV-related LYG as demonstrated in this case, we recommend testing for EBV in CSF for all patients with suspected CLIPPERS. An early referral for brain biopsy and treatment with rituximab should be considered for patients with suspected CLIPPERS who test positive for EBV in their CSF.

GRANULOMATOSE LINFOMATOIDE

2018 ◽  
Vol 5 (2) ◽  
pp. 47-49
Author(s):  
Raissa Chagas Seraiedine ◽  
Monise Marques Mori ◽  
Lucinda Calheiros Guimarães
Keyword(s):  
B Cell ◽  
Epstein Barr Virus ◽  
Diffuse Alveolar Damage ◽  
Pulmonary Nodules ◽  
Lymphocytic Infiltration ◽  
Vascular Wall ◽  
Lymphomatoid Granulomatosis ◽  
Female Ratio ◽  
Barr Virus ◽  
Epstein Barr

ABSTRACT Lymphomatoid granulomatosis (LYG) is a rare extranodal Epstein-Barr virus (EBV) associated B-cell lymphoproliferative disorder. Most cases occur between the fourth and sixth decades of life and the male-to-female ratio is 2:1. The lung is the most frequently involved organ. Radiographically, pulmonary disease is characterized by multiple variable size nodules. Histologically, the lesions show angiocentricity, are rich in T cells, have large atypical B cells, lymphocytic infiltration of the vascular wall and a variable necrosis. LYG is a distinct entity that can usually be differentiated from other EBV-associated B-cell lymphoproliferative disorders on the basis of the combination of clinical presentation and histology. The authors described the case of a 53-year-old woman who was previously diagnosed with sarcoidosis, histoplasmosis, and later with non-Hodgikin B Lymphoma. The lungs presented diffuse alveolar damage, exsudative phase, and infiltration by lymphomatoid granulomatosis. The patient became unstable and died. Grading of these lesions is important because it dictates the treatment choice. Keywords: Lymphomatoid granulomatosis, Epstein-Barr virus, pulmonary nodules.

Three Rwandan Children With Massive Splenomegaly and Epstein-Barr Virus–associated Lymphoproliferative Disorders

2016 ◽  
Vol 38 (5) ◽  
pp. e158-e161 ◽  
Author(s):  
DeAnna Friedman-Klabanoff ◽  
Allison Ball ◽  
Samuel Rutare ◽  
Natalie McCall ◽  
Douglas P. Blackall
Keyword(s):  
Epstein Barr Virus ◽  
Lymphoproliferative Disorders ◽  
Massive Splenomegaly ◽  
Barr Virus ◽  
Epstein Barr

scholarly journals Transcriptomic Abnormalities in Epstein Barr Virus Associated T/NK Lymphoproliferative Disorders

2019 ◽  
Vol 6 ◽  
Author(s):  
Sanjay de Mel ◽  
Joshua Zhi-Chien Tan ◽  
Anand D. Jeyasekharan ◽  
Wee-Joo Chng ◽  
Siok-Bian Ng
Keyword(s):  
Epstein Barr Virus ◽  
Lymphoproliferative Disorders ◽  
Barr Virus ◽  
Epstein Barr

scholarly journals Epstein-Barr virus associated B cell lymphoproliferative disorders following bone marrow transplantation

Blood ◽  
1988 ◽  
Vol 71 (5) ◽  
pp. 1234-1243 ◽  
Author(s):  
RS Shapiro ◽  
K McClain ◽  
G Frizzera ◽  
KJ Gajl-Peczalska ◽  
JH Kersey ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Bone Marrow Transplantation ◽  
B Cell ◽  
Marrow Transplantation ◽  
Cytogenetic Analysis ◽  
Epstein Barr Virus ◽  
Lymphoproliferative Disorders ◽  
Solid Organ ◽  
Barr Virus ◽  
Epstein Barr

Abstract B cell lymphoproliferative disorders (BLPD) developed in eight patients following bone marrow transplantation (BMT) for leukemia (five patients) or immunodeficiency (three patients). Recipients of T depleted marrow from a mismatched donor were at particularly high risk of this complication. Six of 25 (24%) recipients of mismatched T depleted bone marrow developed BLPD. In contrast, none of 47 matched T depleted transplants, one of ten (10%) who received non-depleted marrow from an unrelated donor, and only one of 424 matched non-depleted transplants were associated with BLPD. Epstein-Barr virus (EBV) specific serology and DNA hybridization studies demonstrating five to 50 copies of EBV genome/cell in involved tissues implicate this virus as an associated etiologic agent. Restriction fragment length polymorphism (RFLP) and cytogenetic analysis of involved tissue demonstrated donor origin (five of seven) or host origin (two of seven). Histologic appearance was similar to EBV-induced polymorphic B cell proliferations described following solid organ transplantation, or which occur de novo in primary immunodeficiency. Six of seven patients with adequate tissue available for study were found to have monoclonal proliferations by: in situ immunofluorescence (six of seven), and/or immunoglobulin gene rearrangement, (four of six). Cytogenetic analysis of involved tissues from four patients showed a normal karyotype, whereas two had multiple clonal chromosomal abnormalities. Seven patients died despite aggressive attempts at therapy with combinations of antiviral, immunologic, and chemotherapeutic agents.

scholarly journals Epstein-Barr virus–associated lymphoproliferative disorders

2007 ◽  
Vol 38 (9) ◽  
pp. 1293-1304 ◽  
Author(s):  
Sherif A. Rezk ◽  
Lawrence M. Weiss
Keyword(s):  
Epstein Barr Virus ◽  
Lymphoproliferative Disorders ◽  
Barr Virus ◽  
Epstein Barr
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