Von Willebrand disease - detection, diagnostics and treatment

Abstract

von Willebrand disease is the most common inherited bleeding disorder, with mucocutaneous bleeding and menorrhagia as leading clinical manifestations. Cause of the bleeding diathesis is deficit or dysfunction of the vonWillebrand factor, plasma protein with important roles in adhesion of platelets to the site of vascular injury and transport and protection of coagulation factor VIII. Prevalance of the disease, according to different registries, is between 1 von Willebrand disease on 100 persons to 1 von Willebrand disease on 10000 persons. These data shows that detection of the disorder is not easy and that many cases are undiagnosed. That why bleeding assessment tools are developed and they are widely used for many years. Confirmation of the diagnosis is through laboratory testing. Some of tests are not easily accessible. Therapy of the disorder depends on goals of the treatment (stopping bleeding, prophylaxis or preoperative management). Last year, new guideline for diagnostics and treatment of von Willebrand disease was published by experts of World Federation of Haemophilia, International Society of Thrombosis and Haemostasis, American Society of Hematology and National Hemophilia Foundation. In this paper, new recommendations for detection, diagnostics and treatment of the von Willebrand disease, are presented.