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.