Vaskulárna medicína 3/2016

Von Willebrand disease – prophylaxis and treatment

Inherited von Willebrand disease (vWD) and acquired von Willebrand syndrome (AvWS) are both bleeding disorders caused by deficiencies and/or defects in von Willebrand factor (vWF). DDAVP (1-deamino-8-D-arginin-vasopressin) is favored as main therapy of bleeding for type 1 vWD and it can also be trialed for some patients with types 2A, 2M, and 2N inherited vWD. VWF/factor VIII (FVIII) concentrates provide the main form of therapy in type 2B and type 3 vWD. Therapy for bleeding issues in patients with AvWS is similar to that of vWD. In contrast to hemophilia, prophylaxis is not very common in patients with inherited vWD.

Keywords: von Willebrand disease (vWD), acquired von Willebrand syndrome (AvWS), von Willebrand factor (vWF), coagulation factor VIII (FVIII), 1-deamino-8-D-arginin-vasopressin (DDAVP), replacement therapy