Von Willebrand disease - the 'Dos' and 'Don'ts' in surgery.
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.
Haemophilia Centre, Medical Clinic II, Institute of Transfusion Medicine, Goethe University Hospital, Frankfurt am Main, Germany.
Wolfgang
W
Miesbach
Centre for Thrombosis and Haemostasis, Skane University Hospital, Lund University, Malmö, Sweden.
Erik
E
Berntorp
D000066491:Clinical Decision-Making; D003894:Deamino Arginine Vasopressin / Q000008:administration & dosage / Q000627:therapeutic use; D019468:Disease Management; D005169:Factor VIII / Q000008:administration & dosage / Q000627:therapeutic use; D006470:Hemorrhage / Q000209:etiology* / Q000517:prevention & control*; D006801:Humans; D011292:Premedication; D013514:Surgical Procedures, Operative / Q000009:adverse effects*; D013927:Thrombosis / Q000209:etiology / Q000517:prevention & control; D016896:Treatment Outcome; D014842:von Willebrand Diseases / Q000097:blood / Q000150:complications* / Q000175:diagnosis / Q000628:therapy; D014841:von Willebrand Factor / Q000008:administration & dosage / Q000627:therapeutic use
D014841:von Willebrand Factor; D005169:Factor VIII; D003894:Deamino Arginine Vasopressin
desmopressin; recommendation; surgery; thromboembolic event; von Willebrand disease; von Willebrand factor concentrate