Familial Clustering of Selective IgA Deficiency

Selective IgA deficiency (SIgAD) is the most common primary immunodeficiency but does not always result in clinical disease. This may in part be due to the definition based on serum IgA, while most IgA is secreted at mucosal surfaces, not amenable to measurement. Clinical complications include increased risk of sinopulmonary infections with bacteria and viruses, gastrointestinal infections with a predilection for Giardia lamblia , a myriad of autoimmune diseases including systemic lupus erythematosus, hyper- and hypo-thyroidism, Type 1 diabetes, celiac disease, and rarely, malignancy. SIgAD must be differentiated from IgA deficiency that may be seen with IgG2 or IgG4 deficiency, specific antibody deficiency, or as an early manifestation prior to a diagnosis of common variable immunodeficiency. Secondary IgA deficiency is increasingly recognized and may be due to medications such as anti-epileptics, or antibiotics with disruption of the microbiome which can influence IgA levels, infections or malignancies. Patients with SIgAD should be monitored at regular intervals and educated to be aware of particular complications. There is a rare chance of development of anti-IgA IgE antibodies in patients with complete deficiency, which can result in anaphylaxis if blood products with IgA are administered. Prophylactic antibiotics may be indicated in some cases, and very rarely, supplemental IgG infusions.

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A comprehensive IgA service provided by a blood transfusion center

Munks¹,

Booth²,

Sokol³

1998

Immunohematology

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IgA is best known in transfusion practice for its deficiency when anti-IgA antibodies cause severe anaphylactic reactions. Following the realization that IgA deficient products were needed on demand, blood donors were routinely screened, initially by latex agglutination inhibition and subsequently by hemagglutination inhibition using an Olympus PK-7200™ blood grouping machine. IgA deficiency (<.0016 g/L) was found in 357 (with anti-IgA in 28%) of 301,310 donors, an incidence of 1 in 844. By screening new donors and directed call-up, group O, D-red blood cell (RBC) units are always in stock. During 1 year, the center supplied 79 units of RBCs and 64 units of fresh frozen plasma to a variety of patients with IgA deficiency, including three undergoing liver transplantation. The center also provides a reference service for IgA/anti-IgA status. The technique used (hemagglutination inhibition) has a sensitivity well below the threshold of standard quantitation methods. Samples were most commonly referred from departments investigating possible immunodeficiency and suspected transfusion reactions. Of 247 patients investigated, 122 had IgA deficiency, 43 with anti-IgA (of whom 5 had suffered a transfusion reaction). Donors and patients with anti-IgA were issued blood group cards warning that they should only receive IgA deficient products.

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Familial Clustering of Selective IgA Deficiency

Cited by 5 publications

References 13 publications

Isotype defects

Isotype defects

The clinical implications of selective IgA deficiency

A comprehensive IgA service provided by a blood transfusion center

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