Von Willebrand Disease

Von Willebrand disease is clinically characterized by spontaneous bleeding in the mucous membranes, excessive bleeding wounds, menorrhagia, and bleeding time prolongation with the normal number of platelets. In most cases, von Willebrand disease is transmitted as an autosomal dominant. It is difficult to assess, because in many cases it is a benign clinical manifestations, and diagnosis requires a delicate analysis.

Von Willebrand disease pathogenesis is complex and requires defective platelet function and coagulation mechanisms. Efforts to unravel the complexity has led to a better understanding of the molecular biology of factor VIII, which is a basic glycoprotein coagulant, has three activities net: 1) Factor VIII procoagulant, which depends on the activity of promoting and missing clumps of classic hemophilia as in von Willebrand patients , 2) von Willebrand factor, which is synthesized by endothelial cells and is considered necessary for the adhesion of platelets to subendothelial tissue and in vivo for the conglomerate ristocetin-induced platelet in vitro (ristocetin ancient antibiotics currently used only for studying platelet function), 3) factor VIII-related antigen determinant factor VIII antigen showed discovered by heterologous antibody.

Without going into the complexities of the great, classic and most common variant of von Willebrand disease characterized by proportionate decrease in the three factor VIII activity. In another variant decreased platelet aggregation. Other variants have been described with a defect complex quantitative variables and factor VIII with different mechanisms of inheritance, and several clinical benign.