In addition, concentrations of the procoagulant factors (fibrinogen, von Willebrand factor (vWF) and factor VIII) and fibrinolysis inhibitors (plasminogen activator inhibitor-1 (PAI-1), thrombin activatable fibrinolysis inhibitor (TAFI) and alpha-2-antiplasmin) [2, 4] are increased in active CS (Table 1). This evidence concerns the gene VWF and Cowden syndrome 1.