Congenital von Willebrand Disease-3
Subtype 2A
Autosomal dominant
vWF disease types & subtypes (cont’d):
10 – 20% of cases
vWF susceptible to destruction
Abnormal platelet aggregation
Subtype 2B
Rare mutations results in:
Increased affinity of vWF for gp Ib/IX causes platelet clumping and no vWF for platelet adhesion
“platelet type” or “pseudo-vWF disease” is where gp Ib/IX has increased affinity for normal vWF