Frontiers in Immunology | |
Impaired Control of Epstein–Barr Virus Infection in B-Cell Expansion with NF-κB and T-Cell Anergy Disease | |
Pamela Angelus1  Swadhinya Arjunaraja2  Andrew L. Snow2  Helen C. Su3  | |
[1] Clinical Research Directorate/Clinical Monitoring Research Program, Leidos Biomedical Research, Inc., National Cancer Institute at Frederick, Frederick, MD, United States;Department of Pharmacology and Molecular Therapeutics, Uniformed Services University of the Health Sciences, Bethesda, MD, United States;Laboratory of Clinical Immunology and Microbiology, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD, United States; | |
关键词: Epstein–Barr virus; B-cell expansion with NF-κB and T-cell anergy; CARD11; NF-κB; primary immune deficiency; | |
DOI : 10.3389/fimmu.2018.00198 | |
来源: DOAJ |
【 摘 要 】
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.
【 授权许可】
Unknown