Which of the following is true regarding Von Willebrand disease?
Correct Answer: All of the above.
Description: 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
Category:
Unknown
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