Efficacy, safety and consumption of plasma-derived von Willebrand factor (VWF)/Factor VIII (FVIII) concentrate with 2.4:1 VWF:FVIII ratio for the treatment of von Willebrand Disease: a systematic review

Introduction The therapeutic goal in the management of von Willebrand disease (VWD) is to treat or prevent bleeding events by correcting the deficiency of von Willebrand factor (VWF) and coagulation factor VIII (FVIII). Plasma-derived human coagulation FVIII/human VWF (pdVWF/FVIII; Voncento®​/Biostate®​) is indicated in all age groups for prophylaxis and treatment of haemorrhage or surgical bleeding in patients with VWD when desmopressin is ineffective or contraindicated. A systematic literature review was conducted to evaluate the efficacy, safety and consumption of pdVWF/FVIII in the treatment of patients of all ages with mild, moderate and severe inherited VWD.