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.