Which defects are found in common variable immunodeficiency (CVID)?

Updated: Oct 16, 2018
  • Author: C Lucy Park, MD; Chief Editor: Harumi Jyonouchi, MD  more...
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Answer

Other defects include the following:

  • Reduction in regulatory T cells (Tregs) that is associated with autoimmune manifestation, granulomatous disease, and splenomegaly (Reduced Tregs correlated significantly with the expansion of CD21low B cells.)

  • Reduced expression of markers of Tregs, such as FoxP3, granzyme A, and pStat5 that correlated with the degree of Tregs dysfunction

  • Reduced thymic and bone marrow output, reduced TREC(+) and KREC(+) lymphocytes

  • Lymphopenia and poor lymphocyte proliferation

  • Increased CD8+ cell numbers

  • Lack of antigen-specific T cells

  • Restricted T-cell receptor repertoire

  • Oligoclonal expansion of CD8+ T cells

  • Decreased CD4+ CD45RA+ T cells

  • Decreased production of IL-2, IFN-gamma, IL-4, IL-5, IL-10, and IL-12

  • Impaired T-cell activation

  • Impaired intracellular tyrosine kinase expression

  • Reduced Zap-70 mobilization

  • Deficient CD28 co-signaling

  • Accelerated T-cell death and increased expression of CD95

  • Monocytoid and plasmacytoid dendritic cell defects


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