VON WILLEBRAND'S DISEASE

Question 1

What is vWD?

Answer 1

Autosomal dominant bleeding disorder caused by a deficiency of VWF which is a protein needed to form blood clots

Question 2

What are the 4 types of VWD?

Answer 2

QUANTITATIVE DEFICIENCY - most common; low amounts of VWF causes mild bleeding problems

QUALITATIVE DEFICIENCY - normal VWF levels but doesn’t work properly due to defects

TYPE 2B: overactive VWF bind to platelets leading to thrombocytopenia as they are cleared out

TYPE 3: absence of VWF (most severe)

Question 3

What are the symptoms of VWD?

Answer 3

Bruising 
Epistaxis
Minor wound bleeding
Postoperative bleed
Menorrhagia

Question 4

What are the risk factors of VWD?

Answer 4

FHx

Consanguineous relationship

Question 5

What blood test would you do for VWD?

Answer 5

aPTT: high
PT: normal 
BT: high
FBC: normal (low platelet in 2B)
VWF antigen: low 
Factor VIII activity: low

Question 6

What is the management of VWD?

Answer 6

VWF-containing concentrate

Desmopressin

Tranexamic Acid