von Willebrand disease is the most common hereditary coagulation disorder. It arises from either qualitative or quantitative deficiency of von Willebrand factor, which is a protein that is required for platelet adhesion. There are 4 different types. Type 1 is the most common type. It is an Autosomal Dominant disorder. It decreases the levels of Von Willerbrand factors, which are proteins that are required for platelet adhesion. This decreased level of Von Willerbrand factors is going to result in problems with platelet adhesion.
When an injury occurs the sub-endothelial collagen becomes exposed. This leads to release of Von Willerbrand factors, which act as a linker molecule for platelet to bind to the collagen using Gp1b, which is located on the surface of platelets. Therefor, if there is no Von Willerbrand factors, that initial step for platelet adhesion cannot occur and the patient will not be able to stop bleeding and will present with Mucosal and Skin bleeding.
Treatment of von Willebrand disease focuses on stopping or preventing bleeding episodes, typically by using medications. With the right treatment, most people with von Willebrand disease can lead relatively normal, healthy lives.