A 39-year-old woman with a known history of von Willebrand disease has a ventral hernia after a previous cesarean section and desires to undergo elective repair. Which of the following should be administered preoperatively?
High-Yield Explanation
Von Willebrand disease is similar to true hemophilia in frequency of occurrence. It is being diagnosed more commonly today because of more reliable assays for factor VIII. This autosomal dominant disorder (recessive transmission can occur) is characterized by a diminution in factor VIII: C (procoagulant) activity. The reduction in activity is not as great as in classic hemophilia, and the clinical manifestations are more subtle. These manifestations are often overlooked until an episode of trauma or surgery makes them apparent. Treatment requires correcting the bleeding time and providing factor VIII R: WF (the von Willebrand factor). Only cryoprecipitate is reliably effective. High-purity factor VIII: C concentrates, effective in hemophilia, lack the von Willebrand factor and are consequently undependable