Vaskulárna medicína 1/2021

Successful prophylaxis of bleeding with purified von Willebrand factor concentrate in severe von Willebrand’s disease – personal experience

Von Willebrand’s disease (vWCH) is a congenital bleeding disorder caused by a genetic anomaly in the von Willebrand factor gene characterized by a disorder of structure, function, or a decreased concentration of von Willebrand factor (vWF). A qualitative or quantitative disorder of vWF causes a reduction or loss of the ability of platelets to bind efficiently to injured vascular wall tissue, insufficient protection of plasma coagulation factor VIII (FVIII) from proteolysis, and thus leads to impaired blood clot formation. The aim of the presented article is to provide a brief overview of the importance of vWF in hemostasis, the diversity of clinical manifestations of the disease with emphasis on current options for prophylaxis and treatment of bleeding complications. We present a case report of the patient with severe vWD, in whom we were the first in Slovakia to use in secondary prophylaxis a highly purified plasma vWF concentrate with a low content of FVIII. In such prevention, the patient had a significant reduction in the frequency and intensity of bleeding episodes.

Keywords: von Willebrand disease, plasma-derived vWF concentrate, replacement therapy