Which of the following is true regarding Von Willebrand disease?

Ref. Robbins Pathology. 9th edition. Page. 667   Von Willebrand’s factor is a glycoprotein made in epithelial cells and megakaryocytes which: Acts as a bridging molecule between platelets and the sub-endothelium Helps platelets bind to each other Is a carrier of factor VIII, helping to prevent its breakdown in the circulation    Types of Von Willebrand’s disease: Type 1 Decreased quantity of VWF  Mild to moderate bleeding Type 2 (several subtypes exist)  Qualitative defect - abnormal types of VWF  Type 3  Trace of VWF Autosomal recessive  Severe bleeding disorder Presentation includes Prolonged bleeding after injury  Easy bruising Prolonged epistaxis  Menorrhagia  Oral bleeding (eg after tooth extraction)  Investigation  VWF antigen level At least 2 occasions  Will be low or normal in Von Willebrand’s disease  Note it is elevated by exercise and hyperthyroidism It is decreased by hypothyroidism  Von Willebrand factor ristocetin cofactor activity PT, platelet count and fibrinogen levels are usually normal aPPT usually prolonged Treatment  Desmopressin (DDAVP)  Increases factor VIII and VWF  Useful in type 1, variably useful in type 2, not useful in type 3 Tranexamic acid  An antifibrinolytic agent  Useful in management of epistaxis and menorrhagia  Avoid in patients with history of thromboembolic disease or current upper urinary tract bleeding VWF/factor VIII concentrate