[The management strategy for von Willebrand disease]

The von Willebrand factor (VWF), one of the largest plasma proteins in mammals, is an essential factor in primary hemostasis. It forms a bridge between platelet membrane glycoprotein Ib and extracellular matrix constituents, thereby playing a critical role in platelet adhesion to vascular injury sites. Its deficiency, known as von Willebrand disease is the most common inherited bleeding disorder. VWF is also a carrier protein for blood clotting factor VIII, an interaction required for normal factor VIII survival in circulation. As its symptoms are less severe than those of hemophilia and due to its wide variety, a large number of cases may not have actually been diagnosed. In the selection of therapeutic measures and patient management for bleeding symptoms, introduction of recombinant VWF concentrates is expected to expand the range of therapeutic options.