The Use of Plasma-Derived Concentrates

Von Willebrand disease (VWD) is the most common hereditary bleeding disorder that affects both males and females. It arises from quantitative or qualitative defects of von Willebrand factor (VWF) and causes mucous membrane and soft-tissue bleeding. The aim of treatment is to correct the dual defect of hemostasis caused by the abnormal/reduced VWF and the concomitant secondary deficiency of factor VIII (FVIII). Desmopressin (DDAVP) is the treatment of choice for patients with type 1 VWD who have FVIII and VWF levels of 10 IU/dL or more, while plasma-derived VWF concentrates, either containing FVIII or devoid of this moiety, are indicated for those who are unresponsive or insufficiently responsive to DDAVP (severe type 1, type 2, and type 3 VWD).