Acquired von Willebrand's Syndrome and Ehlers-Danlos Syndrome Presenting with Gastro-Intestinal Bleeding

The clinical and laboratory findings in a patient with severe gastrointestinal bleeding and the Ehlers-Danlos syndrome are described. Coagulation studies and a lack of history of previous haemorrhage were consistent with a diagnosis of acquired von Willebrand's syndrome. His response to treatment with blood transfusion, Factor VIII, cimetidine, tranexamic acid and cyclophosphamide is described. Family studies revealed other members with Ehlers-Danlos syndrome but normal coagulation.