Blood transfusion reaction in a patient with immunoglobulin A deficiency.
Selective deficiency of serum IgA is the most common immunodeficiency in humans; when immunodeficient individuals receive blood transfusions a severe anaphylactoid reaction can develop. The present report describes such a patient. After the transfusion reaction a hemagglutination inhibition assay revealed that her blood contained less than 1.0 micrograms/ml of IgA and an anti-IgA antibody that reacted with the 2 IgA proteins, isotypes IgA1 and IgA2. Immediately after the reaction the patient's serum anti-IgA antibody titer was 1:16,384, and when reevaluated 5 weeks later it was 1:8000. All 3 of her children were shown to be IgA deficient, and 2 of them had antibodies against IgA2. This type of anaphylactoid reaction can be avoided by transfusing blood from IgA-deficient donors, frozen deglycerolized red cells, or red cells that have been washed several times to extract all IgA proteins.
D000328:Adult; D000707:Anaphylaxis / Q000188:drug therapy / Q000209:etiology; D001639:Bicarbonates / Q000627:therapeutic use; D005260:Female; D006801:Humans; D017098:IgA Deficiency; D007153:Immunologic Deficiency Syndromes / Q000235:genetics; D007263:Infusions, Parenteral; D008353:Mannitol / Q000008:administration & dosage; D008776:Methylprednisolone Hemisuccinate / Q000627:therapeutic use; D011247:Pregnancy; D065227:Transfusion Reaction
D001639:Bicarbonates; D008353:Mannitol; D008776:Methylprednisolone Hemisuccinate