Diagnosis and management of von Willebrand disease in Australia
Von Willebrand disease (VWD) is reportedly the most common inherited bleeding disorder, and can also arise as an acquired event where it is termed von Willebrand syndrome. Both arise from deficiency and/or defect of von Willebrand factor (VWF), an adhesive plasma protein that acts primarily to prevent bleeding by anchoring platelets to sites of vascular injury. Factor VIII is also proportionally reduced, due to loss of stabilizing and anti-proteolytic effect normally exerted by VWF on FVIII. Primary VWD management aims to protect against bleeding by replacement of VWF, and sometimes FVIII, using VWF/FVIII concentrates, and/or in some patients, administration of desmopressin (DDAVP) to permit release of endogenous VWF. Adjunct therapies such as anti-fibrinolytics and hormonal therapies in women may also be used, depending on the type and severity of VWD, the type and severity of the bleeding event, and whether therapy is for prophylaxis or treatment. Diagnosis and treatment of VWD involves comprehensive laboratory testing. This review outlines current diagnosis and treatment of VWD in Australia, and is part of an issue of this journal dedicated to diagnosis and treatment of VWD in different geographical localities.