Impaired Control of Epstein–Barr Virus Infection in B-Cell Expansion with NF-κB and T-Cell Anergy Disease

B-cell expansion with NF-κB and T-cell anergy (BENTA) disease is a B-cell-specific lymphoproliferative disorder caused by germline gain-of-function mutations in CARD11. These mutations force the CARD11 scaffold into an open conformation capable of stimulating constitutive NF-κB activation in lymphoc...

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Autores principales: Arjunaraja, Swadhinya, Angelus, Pamela, Su, Helen C., Snow, Andrew L.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2018
Materias:
Immunology
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5809398/
https://www.ncbi.nlm.nih.gov/pubmed/29472930
http://dx.doi.org/10.3389/fimmu.2018.00198
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author Arjunaraja, Swadhinya
Angelus, Pamela
Su, Helen C.
Snow, Andrew L.
author_facet Arjunaraja, Swadhinya
Angelus, Pamela
Su, Helen C.
Snow, Andrew L.
author_sort Arjunaraja, Swadhinya
collection PubMed
description B-cell expansion with NF-κB and T-cell anergy (BENTA) disease is a B-cell-specific lymphoproliferative disorder caused by germline gain-of-function mutations in CARD11. These mutations force the CARD11 scaffold into an open conformation capable of stimulating constitutive NF-κB activation in lymphocytes, without requiring antigen receptor engagement. Many BENTA patients also suffer from recurrent infections, with 7 out of 16 patients exhibiting chronic, low-grade Epstein–Barr virus (EBV) viremia. In this mini-review, we discuss EBV infection in the pathogenesis and clinical management of BENTA disease, and speculate on mechanisms that could explain inadequate control of viral infection in BENTA patients.
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spelling pubmed-58093982018-02-22 Impaired Control of Epstein–Barr Virus Infection in B-Cell Expansion with NF-κB and T-Cell Anergy Disease Arjunaraja, Swadhinya Angelus, Pamela Su, Helen C. Snow, Andrew L. Front Immunol Immunology B-cell expansion with NF-κB and T-cell anergy (BENTA) disease is a B-cell-specific lymphoproliferative disorder caused by germline gain-of-function mutations in CARD11. These mutations force the CARD11 scaffold into an open conformation capable of stimulating constitutive NF-κB activation in lymphocytes, without requiring antigen receptor engagement. Many BENTA patients also suffer from recurrent infections, with 7 out of 16 patients exhibiting chronic, low-grade Epstein–Barr virus (EBV) viremia. In this mini-review, we discuss EBV infection in the pathogenesis and clinical management of BENTA disease, and speculate on mechanisms that could explain inadequate control of viral infection in BENTA patients. Frontiers Media S.A. 2018-02-08 /pmc/articles/PMC5809398/ /pubmed/29472930 http://dx.doi.org/10.3389/fimmu.2018.00198 Text en Copyright © 2018 Arjunaraja, Angelus, Su and Snow. http://creativecommons.org/licenses/by/4.0/ This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
spellingShingle Immunology
Arjunaraja, Swadhinya
Angelus, Pamela
Su, Helen C.
Snow, Andrew L.
Impaired Control of Epstein–Barr Virus Infection in B-Cell Expansion with NF-κB and T-Cell Anergy Disease
title Impaired Control of Epstein–Barr Virus Infection in B-Cell Expansion with NF-κB and T-Cell Anergy Disease
title_full Impaired Control of Epstein–Barr Virus Infection in B-Cell Expansion with NF-κB and T-Cell Anergy Disease
title_fullStr Impaired Control of Epstein–Barr Virus Infection in B-Cell Expansion with NF-κB and T-Cell Anergy Disease
title_full_unstemmed Impaired Control of Epstein–Barr Virus Infection in B-Cell Expansion with NF-κB and T-Cell Anergy Disease
title_short Impaired Control of Epstein–Barr Virus Infection in B-Cell Expansion with NF-κB and T-Cell Anergy Disease
title_sort impaired control of epstein–barr virus infection in b-cell expansion with nf-κb and t-cell anergy disease
topic Immunology
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5809398/
https://www.ncbi.nlm.nih.gov/pubmed/29472930
http://dx.doi.org/10.3389/fimmu.2018.00198
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