Abstract

Background Von Willebrand s disease (VWD) is the most common congenital disorder of hemostasis, characterized by deficient or defective von Willebrand factor. Patients are treated by intravenous replacement of factor VIII/VW (FVIII/ VW) when needed, for prophylaxis before surgical procedures. Anaphylactic reactions to FVIII/VW are rare and desmopressin (DDAVP) can be used as an alternative.

Highlights

  • Von Willebrands disease (VWD) is the most common congenital disorder of hemostasis, characterized by deficient or defective von Willebrand factor

  • Patients are treated by intravenous replacement of factor VIII/VW (FVIII/ VW) when needed, for prophylaxis before surgical procedures

  • Materials and methods We report a case of a 16-year-old female, with VWD and no personal or family history of atopic disease, that was referred to our unit after 4 episodes of facial, upper and lower extremities urticaria, 30 minutes after infusion of FVIII/VW

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Summary

Background

Von Willebrands disease (VWD) is the most common congenital disorder of hemostasis, characterized by deficient or defective von Willebrand factor. Patients are treated by intravenous replacement of factor VIII/VW (FVIII/ VW) when needed, for prophylaxis before surgical procedures. Anaphylactic reactions to FVIII/VW are rare and desmopressin (DDAVP) can be used as an alternative

Materials and methods
Results
Conclusions
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