AUTHOR=Arjunaraja Swadhinya , Angelus Pamela , Su Helen C. , Snow Andrew L. 

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

JOURNAL=Frontiers in Immunology

VOLUME=9

YEAR=2018

URL=https://www.frontiersin.org/journals/immunology/articles/10.3389/fimmu.2018.00198

DOI=10.3389/fimmu.2018.00198

ISSN=1664-3224

ABSTRACT=<p><italic>B</italic>-cell <italic>e</italic>xpansion with <italic>N</italic>F-κB and <italic>T</italic>-cell <italic>a</italic>nergy (BENTA) disease is a B-cell-specific lymphoproliferative disorder caused by germline gain-of-function mutations in <italic>CARD11</italic>. 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.</p>