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Severe combined immunodeficiency disorder
3%
9/312
Bruton's agammaglobulilnemia
5%
17/312
Wiskott-Aldrich syndrome
10%
32/312
IL-12 deficiency
6%
18/312
Selective IgA deficiency
68%
212/312
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This patient is experiencing anaphylaxis. Patients with selective IgA deficiency are predisposed to anaphylactic reactions to blood transfusions. In patients with IgA deficiency, the transfused IgA in the blood products is recognized by the recipient's immune system as a foreign antigen. Selective IgA deficiency is caused by defective heavy chain isotope switching, resulting in a lack of production of IgA. Production of an IgE antibody against the Fc component of the IgA heavy chain can result in anaphylaxis. Washed blood products, that have had all IgA immunoglobulins removed, must be given to these patients. IgA deficient patients can have anaphylaxis at first transfusion by having pre-formed antibodies that suggest a prior unknown or unremembered exposure. Cooper et al. review primary immunodeficiencies. Selective IgA deficiency is relatively common, with an incidence of 1:300-700. The condition may be asymptomatic and often goes undiagnosed until a blood transfusion is needed. Given that IgA acts on mucosal surfaces, patients typically exhibit a history of sinusitis, respiratory tract infections, and gastrointestinal tract infections. The diagnosis is made through low serum IgA levels (below 5 mg/dL) in the setting of normal serum IgG and IgM levels. Yel discusses selective IgA deficiency. Patients should be screened for anti-IgA antibodies in their seurm. Blood products should be prepared from an IgA deficient individual or be saline-washed red blood cells. Concomitant allergic disorders and autoimmune disease are seen in these patients. Illustration A shows the different transfusion reactions that may occur; note that recipient IgA deficiency causes an anaphylactic acute afebrile reaction. Illustration B reviews immunoglobulin class switching; recall that the suspected mechanism of selective IgA deficiency is defective isotope switching. Incorrect Answers: Answers 1,2,3,4: These immune deficiency syndromes, SCID, Bruton's agammaglobulinemia, Wiskott-Aldrich syndrome, and IL-12 deficiency, do not predispose to anaphylaxis with blood transfusion.
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