Von Willebrand Disease

Question 1

What is Von Willebrand Disease (VWD)?

Answer 1

Von Willebrand disease (VWD) is the most common inherited cause of abnormal bleeding (haemophilia). There are many different underlying genetic causes, most of which are autosomal dominant. The causes involve a deficiency, absence or malfunctioning of a glycoprotein called von Willebrand factor (VWF). There are three types based on the underlying cause and ranging from type 1 to type 3. Type 3 is the most severe.

Question 2

Presentation of VWD

Answer 2

Patients present with a history of unusually easy, prolonged or heavy bleeding:

Bleeding gums with brushing
Nose bleeds (epistaxis)
Heavy menstrual bleeding (menorrhagia)
Heavy bleeding during surgical operations

Family history of heavy bleeding or von Willebrand disease is very relevant.

Question 3

Diagnosis of VWD

Answer 3

Diagnosis is based on a history of abnormal bleeding, family history, bleeding assessment tools and laboratory investigations. Due to all the underlying causes there is no easy von Willebrand disease test. This can make diagnosis challenging and beyond the scope of most medical exams.

Question 4

Management of VWD

Answer 4

Von Willebrand disease does not require day to day treatment. Management is required either in response to major bleeding or trauma (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
Factor VIII is often infused along with plasma-derived VWF

Question 5

Women with VWD that suffer from heavy periods can be managed by a combination of:

Answer 5

Tranexamic acid
Mefanamic acid
Norethisterone
Combined oral contraceptive pill
Mirena coil

Question 6

Severe cases:

Answer 6

hysterectomy may be required