Von Willebrands Disease

Question 1

Inheritance?

Answer 1

Autosomal dominant

Question 2

Who is more likely to get it?

Answer 2

Women

O group blood

Question 3

What type of bleeding is seen in vW disease?

Answer 3

Platelet type bleeding

Question 4

What is typical platelet type bleeding?

Answer 4

Submucosal

Question 5

Types of vWD?

Answer 5

Type 1 - quantitative loss

Type 2 - qualitative loss

Type 3 - complete deficiency

Question 6

Where is vWF made and stored?

Answer 6

Made in endothelium

Stored in weibel-palade bodies

Question 7

What is the role of vWF in factor 8 metabolism?

Answer 7

Binds to it to stop it being removed

Without can = factor 8 deficiency

Question 8

Typical presentation?

Answer 8

” 24 y/o woman presents 8 weeks post-partum with heavy vaginal bleeding, fatigue and lightheadedness. She had no problems in pregnancy or during delivery. Uterine tone is normal. No genital tears and no retained placenta. “

Question 9

What is pseudo-vonWillebrands disease?

Answer 9

Caused by lymphoproliferative/myeloproliferative disease

Question 10

What is pseudovWD associated with?

Answer 10

Aortic stenosis

Wilms tumour

Question 11

What is Wilms tumour?

Answer 11

Nephroblastoma

Question 12

TReatment of vWD?

Answer 12

vWF concentrate
DDAVP
Tranexamic acide

Question 13

What is DDAVP and how does it work?

Answer 13

Desmopressin

Short term increases release of vWF from stores (weibel-palade bodies)