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Read moreIt is the most common inherited bleeding disorder
Menorrhagea is a common manifestation of VWD.
VWF serves two roles: (1) as the major adhesion molecule (large VWF multimers) that tethers
the platelet to the exposed subendothelium
(2) as the binding protein for FVIII, resulting in significant prolongation
of the FVIII half-life in circulation.
VWD has been classified into three major types
Type 1 VWD –decrease in VWF protein, VWF function, and FVIII levels
It is the most common type
Type 2 VWD - have functional defects in VWF
Type 3 VWD or severe VWD - virtually no VWF protein and FVIII levels <10%
Investigations
Bleeding time is increased (normal in Hemophilia)
Clotting time is increased
Prolonged PTT
Normal Prothrombin time
Decreased factor VIII
Decreased Ristocetin cofactor ( normal in Hemophilia)
Treatment
1-deamino-8-d-arginine vasopressin (DDAVP, or desmopressin) - usual dose is 0.3 g/kg intravenously or 2 squirts (1 in each nostril) for patients >50 kg (1 squirt for those <50 kg).
VWF replacement in severe cases
Epsilon aminocaproic acid or tranexamic acid - for the prevention or treatment of mucosal bleeding.
Coagulation pathway