Primary immunodeficiency

• Humoural (65%): B cell defects
  • X-linked agammaglobulinemia
  • Common variable immune deficiency (CVID)
  • Transient hypogammaglobulinemia of infancy
  • Hyper-IgM syndrome
  • IgA deficiency
• Cell-mediated (5%): T-cell deficiencies
  • DiGeorge syndrome
  • Chronic mucocutaneous candidiasis
• Combined (15%): B- and T-cell deficiencies, often much more severe
  • Severe combined immunodeficiency disease (SCID)
  • Wiskott-Aldrich syndrome
  • Ataxia-telangiectasia
  • X-linked lymphoproliferative disease
  • CD40 ligand deficiency
• Phagocytic (10%): neutrophil deficiencies
  • Phagocyte deficiencies
  • Chronic granulomatous disease (CGD)
  • Leukocyte adhesion deficiency
• Complement (5%)
  • C2 deficiencies