6. Von Willebrands Disease

Question 1

What is the most common coagulopathy in the world? What percentage of the population does it affect?

Answer 1

Von Willebrands Disease affects 0.5% of the population

Question 2

In simple terms, what is Von Willebrands Disease?

Answer 2

It is when there is either a deficiency, absense or malfunctioning of von Willebrands Factor (VWF)

Question 3

How does someone with Von Willebrands Disease typically present?

Answer 3

• Bleeding gums with brushing
• Nose bleeds (epistaxis)
• Heavy menstrual bleeding (menorrhagia). 1 study showed that 15% of when attending a menorrhagia clinic had undiagnosed von Willebrand’s disease.
• Heavy bleeding during surgical operations
• Family history of heavy bleeding or von Willebrand disease is very relevant.

Question 4

What are the different types of Von Willebrand’s Disease?

Answer 4

• Type 1
• Type 2
• Type 3

Question 5

What is type 1 von Willebrand’s Disease?

Answer 5

Reduced amount of normal vW protein

Question 6

What is type 2 von Willebrand’s Disease?

Answer 6

Abnormal vW protein

In type 2b, this results in the protein being overactive

Question 7

What is type 3 von Willebrand’s Disease?

Answer 7

Little or no vW protein

Question 8

When testing for von Willebrands disease, what are you testing for in the blood?

Answer 8

• Factor VIII
• Von Willebrand’s Antigen
• Von Willebrand’s Activity

Question 9

How can you clinically distinguish between von Willebrands disease type 1 and type 2?

Answer 9

Types 1 and 2 are differentiated by using the ratio of vWF Activity : vWF Antigen.

If the ratio of vWF Activity to vWF Antigen is >0.6 = Type 1

If the ratio of vWF Activity to vWF Antigen is <0.6 = Type 2

Question 10

How is type 1 von Willebrands disease treated?

Answer 10

• Desmopressin (either DDAVP if injection which is prefered, or Minirin if nasal spray) to increase the level of von Willebrands protein, lasting for 24 hours
• Transexamic acid to slow down the dissolution of clots

If you give DDAVP for several consecutive days, there is a chance of a diminishing response. In this event, you can use vWF concentrate

Question 11

How do you treat type 2 von Willebrands Disease?

Answer 11

Type 2 is treated using vWF concentrate, unless they have a good DDAVP test, and are not type 2b, in which case they can be given DDAVP cover just like for type 1.

Question 12

How do you treat type 2b von Willebrands Disease?

Answer 12

Avoid the use of desmopressin (DDAVP) and instead use vWF concentrate

Question 13

How do you treat type 3 von Willebrands Disease?

Answer 13

Always use vWF concentrate

Question 14

How is von Willebrands Disease diagnosed?

Answer 14

• Personal History of Bleeding
• Family History of Bleeding
• Supportive Laboratory Tests

However, there is no easy test for von Willebrands Disease

Question 15

How is von Wilebrands Disease managed?

Answer 15

Management is required in either response to major trauma/bleeding (to stop bleeding) or in preparation for operations (to prevent bleeding).

• Desmopressin can be used to stimulates the release of VWF
• VWF can be infused if needed
• Factor VIII is often infused along with plasma derived VWF

Question 16

How are women that suffer from menorrhagia alongside von Willebrands disease managed?

Answer 16

A combination of

• Tranexamic acid
• Mefenamic acid
• Norethisterone
• Combined oral contraceptive pill
• Mirena coil

Hyssterectomy may be required in severe cases

Iron supplements should also be considered, as they may be losing too much