Definitive

Male or female patient greater than 4 years of age who has a serum IgA of less than 7 mg/dl (0.07 g/L) but normal serum IgG and IgM, in whom other causes of hypogammaglobulinemia have been excluded (see Table). These patients have a normal IgG antibody response to vaccination.

Probable

Male or female patient greater than 4 years of age who has a serum IgA at least 2 SD below normal for age but normal serum IgG and IgM, in whom other causes of hypogammaglobulinemia have been excluded (see Table). These patients have a normal IgG antibody response to vaccination.

Spectrum of disease

Patients with IgA deficiency have an increased incidence of upper respiratory tract infections, allergies and autoimmune disease. Many individuals with IgA deficiency are asymptomatic. Others have persistent or recurrent infections and some develop CVI over time.

Further information

• Patient and Family Handbook For The Primary Immune Deficiency Diseases by IDF.
• Selective IgA Deficiency by The Jeffrey Modell Foundation (JMF).