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Pre-transfusion acetaminophen
0%
0/31
Pre-transfusion diphenhydramine
3%
1/31
Administering type-specific blood
6%
2/31
Administering IVIG with transfusion
Administering washed blood products
81%
25/31
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Given his history of recurrent sinopulmonary infections and his anaphylactic transfusion reaction, this patient is likely IgA deficient. To prevent transfusion reactions, the IgA deficient patient must be given washed blood products without IgA. IgA deficiency is the most common congenital immunodeficiency. IgA deficiency is caused by a defect in the differentiation of B cells, which should otherwise synthesize IgA. The overall prevalence is 1 in 500 people. Most IgA deficient individuals have no symptoms. Of those that are symptomatic, patients may experience recurrent sinopulmonary infections (sinusitis, otitis, bronchitis, and pneumonia), often involving S. pneumoniae. Sharma et al. review IgA transfusion reactions. IgA transfusion reactions are type 1 hypersensitivity (allergic) reactions, which can be mild or severe (anaphylaxis). In more severe reactions, patients may become hypotensive and may develop bronchospasm and stridor. For the reaction to occur, patients must have been pre-sensitized through some prior IgA exposure to create anti-IgA antibodies. To prevent transfusion reactions in IgA deficient individuals, special blood products that are "washed" to remove plasma containing IgA should be used. Hirayama discusses the prevention and management of allergic transfusion reactions. Patients with IgA deficiency can be made to develop tolerance to IgA over time, following repeated administration of IgA-depleted IVIG preparations. For patients having an allergic reaction, diphenhydramine can be used for uticarial reaction, with methylprednisolone available for more severe reactions. For reactions with hypotension and impaired oxygenation, epinephrine and advanced airway management may be utilized. Incorrect Answers: Answers 1-2: Studies have not shown that acetaminophen or diphenhydramine given prophylactically reduce the incidence of transfusion reactions. Answer 3: Failing to administer type-specific blood leads to an acute hemolytic reaction. This would not occur with O-negative blood. Answer 4: Using IVIG depleted of IgA can lead to tolerance to IgA, but such tolerance requires repeated administration of IVIG over many months.
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