Von Willebrand’s Disease Flashcards
What is von Willebrand’s disease?
The most common inherited bleeding disorder.
What is von Willebrand’s disease the most common cause of?
Abnormal bleeding (haemophilia)
What is the genetic inheritance of von Willebrand’s?
Autosomal dominant
How does a patient with Von Willebrand’s Disease typically present?
A history of unusually easy, prolonged or heavy bleeding:
What are the features of Von Willebrand’s Disease?
- Bleeding gums with brushing
- Nose bleeds (epistaxis)
- Heavy menstrual bleeding (menorrhagia)
- Heavy bleeding during surgical operations
- Excess or prolonged bleeding from minor wounds
What is relevant in a patients history with von Willebrand disease?
Family history of heavy bleeding or von Willebrand disease
Who is more likely, men or women, to experience the symptoms of von Willebrand disease?
Women
How many types of Von Willebrand’s disease are there?
3 types
What is Type 1 of Von Willebrand’s disease due to?
Normal molecule but not enough of it
It is caused by a partial quantitative deficiency in VWF
How many different versions of type 2 of Von Willebrand’s disease are there?
4
A, B, N. M
What is Type 2 Von Willebrand’s disease due to?
Abnormal form of vWF
What is Type 2 A of Von Willebrand’s disease due to?
Type 2A VWD is caused by defective platelet adhesion due to decreased high molecular weight VWF multimers
What is Type 2 B of Von Willebrand’s disease due to?
Type 2B is characterised by a pathological increase of VWF-platelet interaction
What is Type 2 N of Von Willebrand’s disease due to?
Type 2N is caused by abnormal binding of the VWF to Factor VIII
What is Type 2 M of Von Willebrand’s disease due to?
Type 2M is caused by a decrease in VWF-platelet interaction
What is Type 3 Von Willebrand’s disease due to?
Caused by almost a complete deficiency of vWF
Autosomal recessive inheritance
Rare
Most severe form
What are the 2 main investigations for Von Willebrand’s disease?
Prolonged bleeding time
Prolonged ATPP
What are some other investigations you can do for Von Willebrand’s disease?
- Factor VIII levels may be moderately reduced
* Defective platelet aggregation with ristocetin
Does Von Willebrand’s require everyday treatment?
No
Only treat after major bleeding or trauma or in preparation for an operation
What is the main management for von Willebrand’s disease?
Desmopressin (DDAVP)
What is the function of desmopressin?
Stimulates the release of VWF (type 1)
What are the other possible treatments for von Willebrand’s disese?
- Factor 8 concentrate
* Tranexamic acid for mild bleeding
What is the medical management for women who suffer heavy periods?
- Tranexamic acid for mild bleeding
* Hysterectomy may be required in severe cases
What is von Willebrand factor?
Large glycoprotein
role: platelet adhesion and carries factor 8
What is an example of how von Willebrand’s would present in a teenage girl?
Heavy periods
Prolonged bleeding time
Prolonged AATP
