Adaptive Immune System

  • ADAPTIVE immune system
  • T-Cell
    • Impaired Development (SCID, Digeorges syndrome)
    • Impaired Maturation, Functioning 
      • SCID, 
      • Hyper-IgE or JOBS syndrome (STAT3 mutation)
      • Wiskott-Aldrich, Ataxia-Telangiectasia)
      • Mechanism of T-cell mediated Immune system
        • Th1 (interferon-γ–producing lymphocytes and IL 12)
          • Control Intracellular Infection
        • Th2 (interleukin-4–producing lymphocytes and TGF B) 
          • Direct antibody-mediated protection against extracellular infection
          • Hyper Ig E syndrome 
        • Th17 - 
          • In response of infection (like yeastB-glucan binding to dectin-1) transforms CD4+ T cell to Th17 by a STAT 3 dependent process
          • Th17 cell procudes IL -22 and IL-17. Together, IL-22 and IL-17 work to generate DEFENSINS in the epithelial tissue 
          • STAT 3 mutation can lead to the defective interaction causing decreased production of DEFENSINS
          • Defect in Leucocyte activation in the epithelium mediated through IL-17 leads to mucocutaneous infection
J Allergy Clin Immunol 2010;125:S33-40. 



B cell differentiation and related primary immunodeficiencies (PIDs) Harrison's Principles of Internal Medicine, 19e

    Selective Antibody Deficiency

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