Hemophilia B. Hemophilia B is produced by a defect in factor IX activity. It is an inherited trait that is found almost exclusively in men. Other hereditary bleeding disorders may occur due to deficiencies or defects in other specific clotting factors. They are diagnosed by specific laboratory studies and treated by factor replacement. Deficiencies in other factors, such as V, VII, X, XI, and XIII, are rarer than the hemophilias, but produce similar symptoms.
Diagnosis depends on detection of bleeding, family history, and laboratory testing.
Hereditary von Willebrand's Disease. In von Willebrand's disease, there is a defect in the body's ability to produce vWf. This results in excessive bleeding from the body cavities, such as the nose, the uterus, the bladder, and the rectum. Most patients with von Willebrand's disease have a history of the disorder in their family. Diagnosis is made based on patient and family history, physical examination, and laboratory testing.