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.
Highlights
Epstein–Barr virus (EBV) is a ubiquitous human herpesvirus that establishes life-long infection in ~90% of individuals [1]
We focus our attention on the incidence and severity of EBV infection in a recently characterized primary immune deficiency (PID) known as B-cell expansion with NF-κB and T-cell anergy (BENTA)
The current cohort of patients remains small, impaired control of EBV infection has emerged as a recurring problem in BENTA disease
Summary
Epstein–Barr virus (EBV) is a ubiquitous human herpesvirus that establishes life-long infection in ~90% of individuals [1]. Constitutive, canonical NF-κB activity induced by GOF CARD11 signaling in B cells drives excessive B cell accumulation in BENTA patients and may predispose them to malignant transformation as additional mutations are acquired over time. Constitutive NF-κB activity in BENTA patient B cells could better enable EBV to expand the pool of latently infected B cells.
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