Von Willebrand Disease: The diagnosis and management of this bleeding disorder

Abstract

von Willebrand disease (vWD) is one of the most common bleeding disorders, first described by Erik von Willebrand in the Aland Islands. It occurs as a result of decreased or abnormal von Willebrand factor (vWF), a factor that is needed in the process of blood coagulation; acting like a “glue” helping platelets to stick together and form the blood clot. The principal function of vWF is binding with other clotting factors (especially Factor VIII) that are also necessary in the clotting process. There are two main forms of this disease: the inherited and the acquired forms; each one's pathophysiology depends on the qualitative or the quantitative defects in vWF. The diagnosis of vWD depends on several factors: measurement of vWD antigen, vWF activity assay, the activity of Factor VIII, and some other additional tests. The management of this disease includes replacement therapy, nonreplacement therapy, and some other kinds of managements that include the use of fibrinolytics and topical agents.