Despite popular belief, there is a rare type of hemophilia that affects women of childbearing age. Von Willebrand disease is the most common of the hereditary bleeding disorders and can affect males and females alike. It results from a factor VIII deficiency and platelet dysfunction. Although factor VIII levels increase naturally during pregnancy, there is an increased risk for postpartum hemorrhage from birth until 4 weeks after delivery as levels of von Willebrand factor (vWf) and factor VIII decrease. The treatment that should be considered first for the patient with von Willebrand disease who experiences a postpartum hemorrhage is:

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Desmopressin, also known as DDAVP, is the first-line treatment for postpartum hemorrhage in patients with von Willebrand disease. It stimulates the release of stored vWf from endothelial cells, thus increasing vWf and factor VIII levels, and promoting platelet adhesion and aggregation. Cryoprecipitate, which contains high levels of vWf and factor VIII, and factor VIII and vWf concentrate are other treatment options, but they carry the risk of transmitting blood-borne infections and developing antibodies against factor VIII or vWf. Hemabate, a synthetic prostaglandin, is used to control postpartum bleeding due to uterine atony, but it is not indicated for bleeding disorders.

Despite Popular Belief, There Is a Rare Type of Hemophilia