Von Willebrand disease

Question 1

Define Von Willebrand disease

Answer 1

Most common bleeding disorder, either qualitative or quantitative disorder of vWF -which is crucial for initial coagulation

Question 2

Aetiology and risk factor of Von Willebrand disease

Answer 2

Usually due to a mutation of vWF-either leading to the vWF not being produced (Type 3), or issues with its function (type 1, 2)
30% of vWD is type 1
Heterozygous are asymptomatic

Risk factors
Lower in blood group O
Black>white
FHx
cosanguinous relations

Question 3

Epidiemology of Von Willebrand disease

Answer 3

Most common bleeding disorder-
affecting 100 per million
maybe even prevalence of 1%

Affects both genders, but women more often diagnosed
75% is type 1

Question 4

Signs and Sx of Von Willebrand disease

Answer 4

Excessive bleeding from minor wounds 
Excessive period bleed
easy/excessive bruising
mucosal bruising (heamorragic bullae)
-this is usually all type1 get, and later in life

occasional signs and sx of anaemia-

type 2/3 can get extra:
and often earlier in life

GI bleeds-malena

in very sevre-CNS bleeds, heamaturia, heamarthrosis

Question 5

Investigations of Von Willebrand disease

Answer 5

FBC-usually normal in FBC

PT-within normal range
aPTT-prolonged

Vonwillibrand assay
Borderlines values (40-60%)-not diagnostic-probably type 1
vWF function assay-lowered

FVII activity-decrease but normal