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Table 16-13

TypePathophysiologyComments
Type 1Partial quantitative deficiency of vWFMildest; most common; responds to DDAVP
Type 2ADysfunction in platelet adhesionMay respond to DDAVP
Type 2MDysfunction in platelet adhesionMay respond to DDAVP
Type 2BIncreased platelet binding affinityThrombocytopenia with DDAVP
Type 2NDecreased FVIII binding affinityOften confused with hemophilia A
Type 3Severe quantitative deficiency of vWFRarest; most severe; usually requires factor concentrates

DDAVP = 1-damino-8-d-arginine vasopressin; FVIII = factor VIII; vWF = von Willebrand factor.