부산대학교 도서관

Von Willebrand's disease (VWD) is the most common hereditary blood-clotting disorder caused by a deficiency of von Willebrand Factor (VWF) and characterized by a defective platelet adhesion and aggregation.1 Replacement therapy with plasma-derived von Willebrand factor-containing factor VIII concentrate (pdVWF/FVIII) is indicated as treatment of choice for surgical and invasive procedures in adult and paediatric patients with severe VWD, when desmopressin (DDAVP) is either ineffective or contraindicated.2,3 The presence of functional VWF is estimated by the VWF:RCo/FVIII:C ratio and may vary among pdVWF/FVIII concentrates.4 Fanhdi SP ® sp (Instituto Grifols S.A., Barcelona, Spain) is a pdVWF/FVIII concentrate characterized by a high content in VWF, with a VWF:RCo/FVIII:C ratio of 1.58±.20.5 Clinical efficacy of Fanhdi SP ® sp has been assessed extensively in VWD patients, to prevent recurrent bleedings in the management of surgery.5-7 However, the available data is retrospective and the only prospective study supporting its use reported interim results only.8 The preliminary interim analysis showed that Fanhdi SP ® sp could be successfully used as a replacement therapy in VWD patients requiring prophylactic treatment during surgeries.8 We report here the final efficacy and safety analysis of this multicentre prospective clinical trial. During the post-surgery period, minor surgeries were treated with 30.6 (19.6-41.7) IU/kg FVIII and 43.6 (25.6-61.7) IU/kg VWF:RCo; and major surgeries with 245.0 (12.5-520.7) IU/kg FVIII and 416.9 (24.6-924.1) IU/kg VWF:RCo. Efficacy and safety evaluation of Fanhdi®, a plasma-derived factor VIII/ von Willebrand factor concentrate, in Von Willebrand's disease patients undergoing surgery or invasive procedures: A prospective clinical study Successful treatment for patients with von Willebrand disease undergoing urgent surgery using factor VIII/VWF concentrate (Humate-P). [Extracted from the article]