von Willebrand Disease

Question 1

What is von Willebrand Disease?

Answer 1

A genetic disorder caused by having a defective or missing clotting protein (von Willebrand Factor)

Question 2

What is von Willebrand Factor?

Answer 2

vWF binds to Factor VIII. A key protein that helps form the platelet plug during the clotting process.

Question 3

What are the three types of von Willebrand Disease (vWD)?

Answer 3

1. Type I - Quantitative Deficiency
2. Type II - Qualitative Deficiency [A,B,M,N] - Determined by the site of mutation in relation to vWF function.
3. Type III - Severe Deficiency [complete vWF]

Question 4

What are the symptoms of von Willebrand Disease? (5)

Answer 4

• Bruising Easily
• Bleeding from your gums/nose
• Heavy / Prolonged bleeding from cuts
• Heavy bleeding during mensturation
• Menorrhagia (abnormally heavy period)

Question 5

What do we look for in blood investigations for von Willebrand Disease? (4)

Answer 5

• Raised APTT (increased clotting time)
• Raised bleeding time
• Reduced vWF Antigen (reduced clotting)

Question 6

What is APTT?

Answer 6

Activated Partial Thromboplastin Time

(it characterises coagulation of blood)

Question 7

What is vWF Ag?

Answer 7

von Willebrand Factor Antigen

It is an assay that measures the concentration of vWF in blood

Question 8

What is DDAVP?

Answer 8

Sold as desmopressin:

Protein clotting medication.

Question 9

What treatment is available for von Willebrand Disease? (4)

Answer 9

• Medication to stop bleeding (DDAVP)
• Topical Application
• Orac Contraceptive Pill (for menorrhagia)

There is NO CURE. But medication and lifestyle changes can control is/

Question 10

What drug should people with vWD avoid?

Answer 10

NSAIDs

Question 11

What medications can stop bleeding? (3)

Answer 11

Tranexamic Acid

Desmopressin

vWF Concentrate