Disorders (percentage of all primary immunodeficiencies)Genetic inheritance patternIncidence, if knownSex affectedAge at diagnosis
Disorders of humoral immunity: B-cell differentiation and antibody production (~ 50)
Common variable immunodeficiencyUndeterminedOne case per 10,000 to 50,000 personsBoth>2 years; can be in 20s or 30s
Selective IgA deficiencyUndeterminedAbout one case per 300 to 700 personsBoth>4 years
Bruton's or X-linked agammaglobulinemiaX-linkedUndeterminedMales>6 months
T-cell defects and combined B-cell and T-cell defects (~30)
Severe combined immunodeficiencyX-linkedOne case per 100,000 to 500,000 personsMales<6 months
T-cell deficient, B-cell competentAutosomal recessiveBoth<6 months
T-cell deficient, B-cell deficientAutosomal recessiveBoth<6 months
DiGeorge syndromeAutosomal dominant or spontaneousUndeterminedBoth<6 months
Wiskott-Aldrich syndromeX-linkedUndeterminedMales<6 months
Ataxia-telangiectasiaAutosomal recessiveUndeterminedBoth>5 years
X-linked hyper IgMX-linkedUndeterminedMalesVariable
Phagocytic disorders (~ 18)
Chronic granulomatous diseaseX-linked (70% of cases) or autosomal recessive (22% of cases)One case per 200,000 personsMales > femalesUsually <5 years; diagnosis can be in 20s and 30s
Complement disorders (~ 2)
Complement deficiencies (at least 16 distinct disorders)Autosomal recessive, autosomal dominant, or X-linkedUndeterminedBothAny age