Acquired von Willebrand's syndrome and Ehlers-Danlos syndrome presenting with gastro-intestinal bleeding.
The clinical and laboratory findings in a patient with severe gastro-intestinal 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.
D000368:Aged; D001780:Blood Coagulation Tests; D004535:Ehlers-Danlos Syndrome / Q000097:blood / Q000150:complications* / Q000235:genetics; D005260:Female; D006471:Gastrointestinal Hemorrhage / Q000097:blood / Q000209:etiology* / Q000235:genetics; D006801:Humans; D008297:Male; D010375:Pedigree; D014842:von Willebrand Diseases / Q000097:blood / Q000150:complications* / Q000235:genetics