Surgical prophylaxis in von Willebrand's disease: a difficult balance to manage

Von Willebrand disease (VWD) is the most common genetic bleeding disorder with a prevalence of approximately 1-2 percent confirmed in different population studies. The severity of the bleeding tendency is usually proportional to the degree of the VWF defect, although the large majority of cases diagnosed appear to have a mild disease. Patients with VWD may require short- or long-term prophylaxis treatment. Short-term prophylaxis is usually performed to prevent excessive bleeding following surgery or invasive procedures, while long-term prophylaxis may be needed to control recurrent mucosal and joint bleeding complicating the more severe forms of VWD. This review is focused on the current knowledge on replacement treatment for patients with VWD disease undergoing surgical or invasive procedures. On the whole, the published studies document the safety and efficacy of VWF/FVIII concentrates as surgical prophylaxis in VWD patients, in particular of Haemate P, the most widely used VWF/FVIII concentrate due to its high VWF:FVIII ratio. The recent literature data also show that the best management of VWD patients undergoing surgery is that to perform a pharmacokinetic study in order to strictly tailor for each VWD patient loading and maintenance doses of VWF/FVIII concentrates. Furthermore, the same studies underscore that, along with VWF levels, FVIII levels should be monitored in the peri-operative period in order to prevent exposures to high FVII levels, associated with an increased risk of venous thrombosis.