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| Introduction to.. von Willebrand Disease.. |
| and Other Coagulation Disorders..... |
| The bleeding disorder now called haemophilia A or factor VIII deficiency has been known since biblical times. Physicians referred to it in medical literature in 1793. Haemophilia B or factor IX deficiency, on the other hand, was not recognized as a distinct entity until 1952. It is now known account for 20% of all cases of haemophilia. At least eight other coagulation disorders have been identified, most of them in the 20th century and some only within the past 25 years.
Von Willebrand Disease It may surprise some in the haemophilia community to learn than von Willebrands disease is believed to be the most common inherited bleeding disorder in humans, estimated to occur in up to 3% of the population. It was first described in 1926 by Erik von Willebrand, a Finnish physician, who reported a new type of bleeding disorder among the inhabitants of some islands between Sweden and Finland. Von Willebrand observed that these patients had an abnormality in their blood platelet function. Years later it was found that some people with von Willebrands disease also have a low level of factor VIII. |