CASE REPORT: Severe and Relapsing Upper Gastrointestinal Bleeding in a Patient with Glanzmann's Thrombasthenia

2000 
Glanzmann’s thrombasthenia (GT) is a rare familial thrombocytic disease inherited as autosomal recessive disorder that can induce hemorrhages. Rarely, these hemorrhages can be fatal. Glanzmann described it for the first time in 1918 (1). In 1974 Nurden and Caen (2) showed that the disease is due to a defect of platelet aggregation, resulting from the absence, or reduced concentration, of the membrane glycoproteic receptor binding the fibrinogen (integrin aIIbb3) (3– 5). The platelet numbers and morphology and tests of coagulation are normal, while the bleeding time is prolonged. Therefore, this disorder is to be suspected in all the patients with: purpura and/or hemorrhage, normal platelets count with absent macroscopic platelet aggregation, and prolonged bleeding time. The molecular aspects of the disease are now well known (6–9), while its clinical features are not yet fully understood (10). We report a case of severe and relapsing upper digestive bleeding in a woman with GT and in which coexisting thrombocytopenia (from HCV-related liver cirrhosis) and H. pylori-positive duodenal ulcer.
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