Table 3.

Illustrative Primary Immunodeficiency Diseases

Disorder of host defense	Disease
Antibody	X-linked agammaglobulinemia
	Common variable immunodeficiency*
	Selective IgA deficiency
	Wiskott-Aldrich syndrome*
Antibody and cell-mediated immunity	Severe combined immunodeficiency
Cell-mediated immunity	Chronic mucocutaneous candidiasis
Phagocytes	Congenital neutropenia
	Chronic granulomatous disease
	Congenital asplenia (Ivemark syndrome)
	Leukocyte adhesion deficiency
	Chediak-Higashi syndrome
Complement	Classical pathway (C1q,r,s; C4; C2; C3 deficiency)
	Alternative pathway (factor D, factor I, factor H, properdin)
	Mannan-binding lectin pathway
	Terminal components (C5, C6, C7, C8, C9)

^*May have associated defects of cell-mediated immunity