Life-threatening reaction to factor VIII concentrate in a patient with severe von Willebrand disease and alloantibodies to von Willebrand factor.
A 16-yr-old girl with severe von Willebrand disease complicated by the development of precipitating alloantibodies to von Willebrand factor (anti-VWF) had a life-threatening anaphylactoid reaction immediately after the infusion of a commercial plasma concentrate of factor VIII/von Willebrand factor. An early post-infusion activation of the complement system was demonstrated by the appearance of C3 split products and by the drop of serum CH50 activity, occurring in parallel with a post-infusion drop in the anti-VWF antibody levels. Immune complexes remained unchanged in the early post-infusion period and rose to a moderate extent only after 24 h. We conclude that biologically active products of the complement system contributed to the onset of this life-threatening reaction which occurred after concentrate infusion.
D000293:Adolescent; D000707:Anaphylaxis / Q000139:chemically induced*; D000936:Antigen-Antibody Complex / Q000032:analysis; D003165:Complement System Proteins / Q000032:analysis; D005169:Factor VIII / Q000627:therapeutic use; D005260:Female; D006801:Humans; D007518:Isoantibodies / Q000096:biosynthesis*; D014842:von Willebrand Diseases / Q000276:immunology* / Q000628:therapy; D014841:von Willebrand Factor / Q000009:adverse effects* / Q000276:immunology / Q000627:therapeutic use
D000936:Antigen-Antibody Complex; D007518:Isoantibodies; D014841:von Willebrand Factor; D005169:Factor VIII; D003165:Complement System Proteins