Von Willebrand disease is inherited in an autosomal dominant…
Von Willebrand disease is inherited in an autosomal dominant manner, equally affecting male and female populations. Bleeding times are typically normal, ristocetin cofactor activity is decreased or normal, and factor VIII is low. All of these disorders are bleeding disorders, each caused by deficiencies of different clotting factors. Hemophilia A occurs when factor VIII is deficient, hemophilia B when factor IX is deficient, and von Willebrand disease when the von Willebrand factor is deficient or abnormal. All are clinically similar, characterized by prolonged or renewed bleeding after injuries, tooth extractions, or surgery. They should be suspected in patients with deep muscle hematomas, intracranial bleeding in the absence of major trauma, unexplained GI bleeding or hematuria, recurrent and bilateral prolonged nosebleeds, and excessive bruising. In severe cases, joint bleeding is a frequent symptom. Age of diagnosis and severity of symptoms depends on the level of normal functioning clotting factor present. Coagulation factor assays are used for diagnosis.