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

B-cell expansion with NF-kappa B 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-kappa B 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.