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

Abstract:

: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.

journal_name

Front Immunol

journal_title

Frontiers in immunology

authors

Arjunaraja S,Angelus P,Su HC,Snow AL

doi

10.3389/fimmu.2018.00198

subject

Has Abstract

pub_date

2018-02-08 00:00:00

pages

198

issn

1664-3224

journal_volume

9

pub_type

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