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Von Willebrand disease – the ‘Dos’ and ‘Don'ts’ in surgery
Authors:Wolfgang Miesbach  Erik Berntorp
Affiliation:1. Haemophilia Centre, Medical Clinic II, Institute of Transfusion Medicine, Goethe University Hospital, Frankfurt am Main, Germany;2. Centre for Thrombosis and Haemostasis, Skane University Hospital, Lund University, Malm?, Sweden
Abstract:Von Willebrand disease (VWD) is the most common genetic bleeding disorder. VWD is caused by a deficiency or dysfunction of von Willebrand factor (VWF), a plasma protein that mediates the initial adhesion of platelets at sites of vascular injury and binds and stabilises coagulation factor VIII (FVIII) in the blood. Prophylaxis of surgical bleeding in patients with VWD requires consideration of the individual situation, including the type of procedure and the bleeding rate, before decisions about treatment type, dose, duration and adjunctive therapy with antifibrinolytics or antithrombotic prophylaxis can be made. Although desmopressin (DDAVP)‐stimulated release of endogenous VWD is an effective treatment strategy in many patients, plasma concentrates containing VWF are the preferred option for most patients undergoing surgical procedures. Recommendations for the management of surgery in patients with VWD are summarised, including the severity of VWD and the type of the surgical procedure.
Keywords:von Willebrand disease  von Willebrand factor concentrate  desmopressin  thromboembolic event  surgery  recommendation
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