Most patients with congenital von Willebrand disease (VWD) undergoing major surgical procedures require prophylactic replacement therapy with von Willebrand factor (VWF)/factor VIII (FVIII) concentrates. Venous thromboembolism has been reported in such patients, as a result of a combination of various treatments (i.e., type of surgery, progressive increase of post-infusion FVIII plasma levels) and/or patient-related thrombotic risk factors. On the whole, the literature data show that venous thromboembolic complications in surgical VWD patients who have been prophylactically treated with VWF/FVIII concentrates are extremely rare. Indeed, only 11 cases have been reported in the literature, mostly occurring during orthopedic procedures. Thus, in absence of a widely accepted consensus and adequate prospective studies, we advise that the need for thromboprophylaxis in such patients should be evaluated individually, after a careful risk/benefit analysis.
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