International survey on the management of IgA deficiency: The BEST Collaborative Study.

BACKGROUND AND

objectivesSelective immunoglobulin A (IgA) deficiency is the most common human immunodeficiency, affecting approximately 1 in 500-800 individuals of European ancestry. It is defined by a serum IgA level below 7 mg/dL, with normal immunoglobulin G (IgG) and immunoglobulin M (IgM) levels. Since 1968, severe allergic transfusion reactions have been reported in IgA-deficient patients, often linked to anti-IgA antibodies. This led to recommendations for using IgA-deficient blood components in affected individuals. However, due to the rarity of such reactions, standardized and evidence-based guidelines are lacking, while blood services face logistical challenges maintaining registries of IgA-deficient donors and inventories of compatible products. MATERIALS AND

methodsAn international survey was conducted to assess current practices in managing IgA deficiency. The survey explored the frequency of transfusion reactions involving IgA/anti-IgA, detection methods for IgA deficiency and anti-IgA antibodies, reasons for requesting IgA-deficient products, product availability and strategies to facilitate access to IgA-deficient products.

resultsParticipants from 14 countries/regions across four continents reported a wide variability in defining IgA deficiency, criteria for recommending IgA-deficient products and anti-IgA testing practices.

conclusionThese findings highlight the need for international reference standards and harmonized recommendations to improve the management of IgA-deficient patients. Establishing harmonized best practices and evidence-based guidelines should enhance patient safety and support clinical decision making globally.