Von Willebrand’s, << von WIHL uh brandz, >> disease is a hereditary disease in which the skin bleeds for an abnormally long period of time when injured. It is similar to the blood disorder hemophilia (see Hemophilia ). However, unlike hemophilia, von Willebrand’s disease causes prolonged external bleeding, and internal bleeding is rare. In addition, most hemophiliacs are males, but von Willebrand’s disease affects both sexes. Von Willebrand’s disease occurs worldwide, but it is most common in the Scandinavian countries. It was first described in 1926 by Erik von Willebrand, a German professor of medicine.
For clotting to occur, blood must contain various substances called clotting factors. Von Willebrand’s disease is caused by a deficiency of one such factor, called the von Willebrand factor. Most patients experience mild bleeding, most commonly from the nose. But some patients bleed severely, especially after having a tooth extracted or following surgery or childbirth. Blood products containing the von Willebrand factor are given intravenously to treat the disease. A drug called desmopressin acetate also has proved effective in treating some patients.
