Clinical Information

IgA, the predominant immunoglobulin secreted at mucosal surfaces, consists of 2 subclasses, IgA1 and IgA2. IgA1 is the major (approximately 80%) subclass in serum. IgA2 is the major subclass in secretions such as milk. Although IgA deficiency is a common defect (1 in 700), it is usually asymptomatic. IgA deficiency with or without IgG subclass deficiency, however, can lead to recurrent pulmonary and gastrointestinal infections. Some infections (eg, recurrent sinopulmonary infections with Haemophilus influenzae) may be related to a deficiency of IgA2 in the presence of normal total IgA concentrations.

Paradoxically, bacterial infections may also cause IgA deficiency. For example, IgA1 (but not IgA2) can be cleaved and inactivated by certain bacteria, thus depleting the majority of the IgA. In the presence of a concurrent IgA2 deficiency, infection by these organisms results in an apparent IgA deficiency.

IgA deficiency is 1 cause of anaphylactic transfusion reactions. In these situations, IgA-deficient patients produce anti-IgA antibodies that react with IgA present in the transfusion product. While transfusion reactions typically occur in patients who have no detectable levels of IgA, they can occur in patients with measurable IgA. In these situations, the complete deficiency of 1 of the IgA subclasses may be the cause of the transfusion reactions.