Von Willebrand Disease

Question 1

What is Von Willebrand disease?

Answer 1

Most common form of haemophilia (abnormal bleeding) due to deficiency in functioning VWF i.e. can be absent or malfunctioning

Question 2

What are the main cause of VWD?

Answer 2

Tends to be autosomal dominant mutation of the gene coding for VWF

Question 3

What are the 2 gross classifications of VWD?

What types are associated with this classifications?

Answer 3

Quantitative
Type 1= partial
Type 3= complete -> SEVERE

Qualitative
Variants of type 2 VWD i.e. 4 variants

Question 4

How might someone with VWD present?

Answer 4

Bleeding gums when brushing teeth
Epistaxis
Menorrhagia
Heavy bleeding during surgical operations
FH of heavy bleeding -> inherited disease

I.e. easy, prolonged and heavy bleeding

Question 5

How is VWD diagnosed?

Answer 5

Family history
History of abnormal bleeding
Bleeding assessment tools
Measure VWF levels= antigen and activity to try and determine the different types
Measure platelets and function to rule out platelet disorder

NOTE:
-can be very challenging to diagnose because there is no simple test for VWD

Question 6

How is VWD managed?

Answer 6

Does not require everyday treatment
Only treated in major bleeding or trauma or in prep for operations

Management in these instances:

• desmopression= stimulates the release of VWF
• VWF= infusion
• Factor VIII= infusion

Question 7

How can menorrhagia due to VWD be managed?

Answer 7

TXA i.e. tranexamic acid

Mefanamic acid

Norethisterone

Combined OCP

Mirena coil

Question 8

What is the MOA of TXA?

Answer 8

Inhibits action of plasminogen to prevent fibrin breakdown
Promotes clotting and downregulates fibrinolysis

Consequences:
-stable clot maintained