Von Willebrands

Question 1

What is Von Willebrand’s disease?

Answer 1

A bleeding disorder caused by a deficiency or dysfunction of von Willebrand factor (vWF)

It affects the ability of blood to clot properly.

Question 2

What characterizes Type 1 Von Willebrand’s disease?

Answer 2

Common form with reduced levels of vWF

It typically presents with mild bleeding symptoms.

Question 3

What is the nature of Type 2 Von Willebrand’s disease?

Answer 3

Uncommon form with abnormal function of vWF

It can lead to variable bleeding patterns.

Question 4

What defines Type 3 Von Willebrand’s disease?

Answer 4

Rare form with near absence of vWF

Patients may exhibit symptoms similar to those with moderate to severe hemophilia.

Question 5

What is a typical bleeding picture in Von Willebrand’s disease?

Answer 5

Typically associated with mild bleeding

Bleeding patterns can vary based on the type.

Question 6

What are the laboratory findings in Von Willebrand’s disease?

Answer 6

• Low: vWF factor
• Low: factor 8 activity
• Prolonged bleeding time
• APTT increased (due to F8)
• PT (INR) normal
• Normal platelet count
• Impaired ristocetin induced platelet aggregation

These tests help in diagnosing the disease.

Question 7

What is the initial treatment for Von Willebrand’s disease?

Answer 7

• TXA 15mg/kg IV
• TXA 25mg/kg PO
• DDAVP 0.3mcg/kg IV/SC (not effective for Type 3)
• VWF / F8 concentrate (Biostate) - 30-60u/kg depending on bleed

Treatment varies based on the severity and type of the disease.