Von Willebrand Disease

Question 1

What is the most common inherited blood clotting abnormality?

1 - Haemophilia A
2 - Haemophilia B
3 - Von Willebrand disease
4 - Vitamin D deficiency

Answer 1

3 - Von Willebrand disease

Question 2

What is the incidence of Von Willebrand disease?

1 - 1000 cases per 100,000
2 - 100 cases per 100,000
3 - 10 cases per 100,000
4 - 1 cases per 100,000

Answer 2

4 - 1 cases per 100,000

Affects men and women equally

Question 3

What age does the incidence of Von Willebrand disease peak?

1 - 20-30
2 - 30-40
3 - 40-50
4 - 50-60

Answer 3

2 - 30-40

Question 4

What is Von Willebrand’s disease?

1 - impaired intrinsic coagulation cascade
2 - impaired extrinsic coagulation cascade
3 - impaired platelet function
4 - low number and/or quality of von willebrand factor

Answer 4

4 - low number and/or impaired function of von willebrand factor

Results in dysfunctional primary haemostasis

Question 5

Once endothelium cells are damaged they release Von Willebrand factor (vWF) that binds to exposed collagen below endothelial cells. The vWF can then bind with what?

1 - GP2b3a on platelets
2 - WBC
3 - RBCs
4 - GP1b on platelets

Answer 5

4 - GP1b on platelets

This then activates platelets and causes primary haemostasis

Question 6

In addition to binding between collagen on sub-endothelial cells with platelets to initiate primary haemostasis, Von Willebrand factor bind with another coagulation factor, ensuring it is not degraded early. Which factor is this?

1 - factor V (5)
2 - factor VI (6)
3 - factor VII (7)
4 - factor VIII (8)

Answer 6

4 - factor VIII (8)

Question 7

Von Willebrand disease can be transmitted via autosomal dominant transmission. What does that mean?

1 - mutated gene is located on X chromosome
2 - mutated gene is located on the Y chromosome
3 - only 1 mutated gene is required on one chromosome
4 - mutated gene needs to be on both X and Y chromosome

Answer 7

3 - only 1 mutated gene is
required on one chromosome

• 1 mutated gene causes phenotype
• can affect men and women equally

Question 8

Von Willebrand disease can be categorised based on the severity and what is affected. These categories are type 1, 2 and 3. What is the abnormality in type 1 VWD?

1 - low levels of VWF
2 - virtually complete deficiency of VWF
3 - qualitative abnormality of VWF
4 - all of the above

Answer 8

1 - low levels of VWF

Function of remaining vWF if normal

• most common form of VWD (75% of cases)
• low number of VWF

Question 9

Von Willebrand disease can be categorised based on the severity and what is affected. These categories are type 1, 2 and 3. What is the abnormality in type 2 VWD?

1 - partial quantitative deficiency of VWF
2 - virtually complete deficiency of VWF
3 - normal levels but impaired function of VWF
4 - low levels and poor function

Answer 9

3 - normal levels but impaired function of VWF

Question 10

Von Willebrand disease can be categorised based on the severity and what is affected. These categories are type 1, 2 and 3. What is the abnormality in type 3 VWD?

1 - partial quantitative deficiency of VWF
2 - virtually complete deficiency of VWF
3 - qualitative abnormality of VWF

Answer 10

2 - virtually complete
deficiency of VWF

• most severe form of the VWD

Question 11

Which of the following clinical symptoms do von Willebrand disease patients NOT commonly present with?

1 - epistaxis (nose bleeds)
2 - menorrhagia
3 - haemarthroses
4 - mucocutaneous bleeds

Answer 11

3 - haemarthroses

• means bleeding into joints like in Haemophilia
• rare, but can occur

Question 12

All of the following are symptoms of von Willebrand disease, but which is most common in women?

1 - menorrhagia
2 - epistaxis
3 - excessive bruising
4 - prolonged bleeding post surgery
5 - bleeding gums

Answer 12

1 - menorrhagia

f left untreated the former may lead to anaemia and iron deficiency

Question 13

When trying to diagnose a patient with von Willebrand disease, what is often the most important question in the history taking?

1 - medication history
2 - medical history
3 - family history
4 - current symptoms

Answer 13

3 - family history

Inherited, so must ask this question

Question 14

When trying to diagnose a patient with von Willebrand disease, do we often see abnormalities in FBC?

Answer 14

• no

FBC is typically normal, BUT in rare cases like vWB type 2, thrombocytopenia (low platelets can occur

Question 15

The activated partial thromboplastin time (APTT) is a measurement of the coagulation cascade and can be normal or prolonged in von Willebrand Disease (vWD) Why would the APTT be increased (slower blood clotting) in a patient with suspected vWD?

1 - measures total coagulation time
2 - measures all factors involved in coagulation cascade
3 - measures platelet clotting ability
4 - measures intrinsic pathway

Answer 15

4 - measures intrinsic pathway

• VWD is associated with factor VIII
• VIII is part of intrinsic coagulation pathway
• would also be affected by the lack or primary haemostasis

Question 16

How is von Willebrand Disease (vWD) diagnosed?

1 - clinical diagnosis
2 - genetic testing
3 - vWB measurement
4 - all of the above

Answer 16

3 - vWB measurement

Question 17

What is the 1st line management for patients with von Willebrands factor?

1 -plasma VIII with intact VWF
2 - factor VIII
3 - desmopressin
4 - ferrous sulphate

Answer 17

3 - desmopressin

Shown to induce the synthesis of vWF by endothelial cells

Question 18

If desmopressin is not effective for the treatment of von Willebrand’s factor (vWF) which of the following should be given to treat patients prophalyaxis?

1 - recumbent VWF
2 - plasma VIII with intact VWF
3 - recumbent factor VIII
4 - recumbent factor VIII and VWF

Answer 18

2 - plasma VIII with intact VWF

Question 19

Which 2 of the following are the 1st line management for patients with von Willebrands factor during active bleeding?

1 - plasma VIII with intact VWF
2 - factor VIII
3 - desmopressin
4 - tranexamic acid

Answer 19

3 - desmopressin
4 - tranexamic acid

If not effective give plasma VIII with intact VWF

Question 20

Patients with von Willebrand Disease (vWD) should be advised to avoid which 2 of the following due to increased risk of bleeding?

1 - opioids
2 - aspirin
3 - paracetamol
4 - NSAIDs

Answer 20

2 - aspirin
4 - NSAIDs

Both increase the risk of bleeding