Type | Pathophysiology | Comments |
---|---|---|
Type 1 | Partial quantitative deficiency of vWF | Mildest; most common; responds to DDAVP |
Type 2A | Dysfunction in platelet adhesion | May respond to DDAVP |
Type 2M | Dysfunction in platelet adhesion | May respond to DDAVP |
Type 2B | Increased platelet binding affinity | Thrombocytopenia with DDAVP |
Type 2N | Decreased FVIII binding affinity | Often confused with hemophilia A |
Type 3 | Severe quantitative deficiency of vWF | Rarest; most severe; usually requires factor concentrates |
DDAVP = 1-damino-8-d-arginine vasopressin; FVIII = factor VIII; vWF = von Willebrand factor.