IgA anaphylactic transfusion reactions are rare events that must be distinguished from more common acute generalized transfusion-related events, including ABO hemolytic reactions, acute hypervolemia, transfusion-related acute lung injury, drug allergy, and other acute adverse reactions. There are no specific clinical findings that establish a diagnosis of IgA anaphylaxis. Laboratory diagnosis requires demonstration of IgA deficiency (<0.05 mg/dl) and the presence of anti-IgA. Clinical management consists of general supportive measures and, when needed, transfusing washed red blood cells, washed platelets, or blood components collected from donors known to be IgA-deficient. IgA-depleted intravenous immune globulin products are available and have been used successfully in patients with primary immunodeficiency disease who have recurrent acute transfusion reactions and who require chronic replacement therapy with intravenous immune globulin. In selected life-threatening emergencies, there may be a greater risk by withholding treatment rather than slowly transfusing conventional blood products in a well-monitored and controlled environment. Support for that approach is derived from published reports of uneventful transfusions of conventional blood components in patients known to have previous adverse transfusion reactions and anti-IgA in their plasma.