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

  1. Author:
    Arjunaraja, Swadhinya
    Angelus, Pam
    Su, Helen C.
    Snow, Andrew L.

  2. Author Address

    Uniformed Serv Univ Hlth Sci, Dept Pharmacol & Mol Therapeut, Bethesda, MD 20814 USA.NIAID, Lab Clin Immunol & Microbiol, NIH, 9000 Rockville Pike, Bethesda, MD 20892 USA.Leidos Biomed Res Inc, Natl Canc Inst Frederick, Clin Res Directorate, Clin Monitoring Res Program, Frederick, MD USA.
    1. Year: 2018
    2. Date: Feb 8
  2. Journal: FRONTIERS IN IMMUNOLOGY
  3. FRONTIERS MEDIA SA,
    1. 9
  5. Type of Article: Review
  6. Article Number: 198
  7. ISSN: 1664-3224
  1. Abstract:

    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.

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External Sources

  1. View Full Text
  2. DOI: 10.3389/fimmu.2018.00198
  3. PMID: 29472930
  4. View record in Web of Science®
  5. WOS: 000424472200001

Library Notes

  1. Fiscal Year: FY2017-2018
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