Von Willebrand’s Disease

Question 1

What is von Willebrand’s disease?

Answer 1

The most common inherited bleeding disorder.

Question 2

What is von Willebrand’s disease the most common cause of?

Answer 2

Abnormal bleeding (haemophilia)

Question 3

What is the genetic inheritance of von Willebrand’s?

Answer 3

Autosomal dominant

Question 4

How does a patient with Von Willebrand’s Disease typically present?

Answer 4

A history of unusually easy, prolonged or heavy bleeding:

Question 5

What are the features of Von Willebrand’s Disease?

Answer 5

• Bleeding gums with brushing
• Nose bleeds (epistaxis)
• Heavy menstrual bleeding (menorrhagia)
• Heavy bleeding during surgical operations
• Excess or prolonged bleeding from minor wounds

Question 6

What is relevant in a patients history with von Willebrand disease?

Answer 6

Family history of heavy bleeding or von Willebrand disease

Question 7

Who is more likely, men or women, to experience the symptoms of von Willebrand disease?

Answer 7

Women

Question 8

How many types of Von Willebrand’s disease are there?

Answer 8

3 types

Question 9

What is Type 1 of Von Willebrand’s disease due to?

Answer 9

Normal molecule but not enough of it

It is caused by a partial quantitative deficiency in VWF

Question 10

How many different versions of type 2 of Von Willebrand’s disease are there?

Answer 10

4

A, B, N. M

Question 11

What is Type 2 Von Willebrand’s disease due to?

Answer 11

Abnormal form of vWF

Question 12

What is Type 2 A of Von Willebrand’s disease due to?

Answer 12

Type 2A VWD is caused by defective platelet adhesion due to decreased high molecular weight VWF multimers

Question 13

What is Type 2 B of Von Willebrand’s disease due to?

Answer 13

Type 2B is characterised by a pathological increase of VWF-platelet interaction

Question 14

What is Type 2 N of Von Willebrand’s disease due to?

Answer 14

Type 2N is caused by abnormal binding of the VWF to Factor VIII

Question 15

What is Type 2 M of Von Willebrand’s disease due to?

Answer 15

Type 2M is caused by a decrease in VWF-platelet interaction

Question 16

What is Type 3 Von Willebrand’s disease due to?

Answer 16

Caused by almost a complete deficiency of vWF
Autosomal recessive inheritance
Rare
Most severe form

Question 17

What are the 2 main investigations for Von Willebrand’s disease?

Answer 17

Prolonged bleeding time

Prolonged ATPP

Question 18

What are some other investigations you can do for Von Willebrand’s disease?

Answer 18

• Factor VIII levels may be moderately reduced

* Defective platelet aggregation with ristocetin

Question 19

Does Von Willebrand’s require everyday treatment?

Answer 19

No

Only treat after major bleeding or trauma or in preparation for an operation

Question 20

What is the main management for von Willebrand’s disease?

Answer 20

Desmopressin (DDAVP)

Question 21

What is the function of desmopressin?

Answer 21

Stimulates the release of VWF (type 1)

Question 22

What are the other possible treatments for von Willebrand’s disese?

Answer 22

• Factor 8 concentrate

* Tranexamic acid for mild bleeding

Question 23

What is the medical management for women who suffer heavy periods?

Answer 23

• Tranexamic acid for mild bleeding

* Hysterectomy may be required in severe cases

Question 24

What is von Willebrand factor?

Answer 24

Large glycoprotein

role: platelet adhesion and carries factor 8

Question 25

What is an example of how von Willebrand’s would present in a teenage girl?

Answer 25

Heavy periods
Prolonged bleeding time
Prolonged AATP