title: Effect of circulating immune complexes on transfusional therapy in patients with hemophilia or von Willebrand's disease.
abstract: Circulating immune complexes were examined in patients with hemophilia or von Willebrand's disease in order to determine the immediate or long-term side effects after transfusion. The conglutinin binding assay which allows quantitation of C3bi-bearing immune complexes was used for 82 patients with hemophilia A. Immune complexes were detected in 37 (45%) of these cases prior to transfusion. Immune complexes also were detected in four of 11 patients with hemophilia A and factor VIII inhibitors, in five of 11 patients with hemophilia B, and in three of 10 patients with von Willebrand's disease. The levels of circulating immune complexes in 21 patients with hemophilia A and seven with von Willebrand's disease significantly increased 24 hours after concentrate or cryoprecipitate transfusions. Purified immune complexes from three patients with hemophilia A were shown to contain IgG, IgM, and complement components. No factor VIII coagulant or antigenic protein or fibrinogen was identified in the immune complexes using specific antisera. Side effects immediately after transfusion were not associated with immune complexes. The levels of factor VIII or IX after transfusion were not particularly decreased in relation to the presence of immune complexes. Finally, the presence of circulating immune complexes in the patients studied did not correlate with the number of transfusions, the units of concentrates injected, the presence of HBsAg or HbsAb, the levels of plasma aspartate transferase, or the presence of rheumatoid factor. Proteinuria was absent in all the patients studied.
mesh_terms: D000936:Antigen-Antibody Complex / Q000032:analysis / Q000502:physiology*; D001219:Aspartate Aminotransferases / Q000032:analysis; D001803:Blood Transfusion; D003176:Complement C3 / Q000032:analysis; D015933:Complement C3d; D005169:Factor VIII / Q000008:administration & dosage / Q000276:immunology; D006467:Hemophilia A / Q000276:immunology / Q000628:therapy*; D006510:Hepatitis B Antibodies / Q000032:analysis; D006514:Hepatitis B Surface Antigens / Q000032:analysis; D006801:Humans; D065227:Transfusion Reaction; D014842:von Willebrand Diseases / Q000276:immunology / Q000628:therapy*
chemical_list: D000936:Antigen-Antibody Complex; D003176:Complement C3; D006510:Hepatitis B Antibodies; D006514:Hepatitis B Surface Antigens; D015933:Complement C3d; D005169:Factor VIII; D001219:Aspartate Aminotransferases