Pathology - von Willebrand Disease
von Willebrand disease (vWD) is the most common inherited bleeding illness. It is autosomal dominant and is most commonly (in vWD Type I) the outcome of a quantitative deficiency in von Willebrand factor (vWF). vWF is a vital component of hemostasis, forming the adhesive bond between platelets and the damaged endothelium, and also serves as a carrier protein for factor VIII. The condition results in prolonged bleeding time on coagulation testing, as faulty platelet adhesion impedes development of the initial platelet plug, and sometimes prolonged PTT, due to its role in factor VIII availability. Abnormal ristocetin cofactor assay or reduced vWF antigen validates the diagnosis. Patients may initially be recognized via operations such as circumcision, dental work, or surgery. Other manifestations may include easy bruising, excessive bleeding with loss of deciduous teeth or delivery, epistaxis, and menorrhagia. Severe bleeding, such as recurrent hemarthrosis in hemophilia, is unusual. Desmopressin (DDAVP) promotes release of vWF and factor VIII and is a mainstay of therapy, with oral contraceptive pills (estrogen also boosts vWF levels) widely used for menorrhagia.
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