title: Successful surgery using recombinant factor VIIa for recurrent, idiopathic nonulcer duodenal bleeding in a patient with Glanzmann's thrombasthenia.
abstract: A 68-year-old man with Glanzmann's thrombasthenia suffered from recurrent cryptogenic bleeding originating in the upper duodenal flexure. Extensive endoscopic procedures and medical treatments were unsuccessful and proximal duodenectomy was proposed. In preceding years platelet transfusions had often had a suboptimal result and were complicated by allergic reactions. Surgery was carried out while he was being treated with recombinant Factor VIIa. Neither major blood loss nor other complications occurred. Histological examination of the bleeding site failed to show abnormalities and the nature of this patient's bleeding problem remained unexplained. Glanzmann's thrombasthenia is a rare autosomal recessive disorder of platelet aggregation characterized by a lifelong bleeding tendency due to abnormalities of the glycoprotein IIb-IIIa membrane complex. Common clinical manifestations include purpuric type bleeding, epistaxis, menorrhagia and gingival bleeding. Spontaneous bleeding is uncommon but posttraumatic and postoperative hemorrhage may be particularly serious. There is no specific treatment. Prophylactic and therapeutic platelet transfusions are the cornerstone of supportive treatment. In many patients the efficacy of this approach is diminished by allo-anti-platelet antibodies. We report on a patient with Glanzmann's disease with recurrent nonulcer duodenal bleeding refractory to conservative medical treatment. Despite documented suboptimal effectiveness of platelet transfusions, he underwent successful surgery with administration of recombinant factor VIIa (rFVIIa).
mesh_terms: D000368:Aged; D004378:Duodenal Diseases / Q000601:surgery*; D005167:Factor VII / Q000627:therapeutic use*; D015942:Factor VIIa; D006471:Gastrointestinal Hemorrhage / Q000601:surgery*; D006801:Humans; D008297:Male; D011994:Recombinant Proteins / Q000627:therapeutic use*; D013915:Thrombasthenia / Q000150:complications* / Q000188:drug therapy
chemical_list: D011994:Recombinant Proteins; D005167:Factor VII; C103587:recombinant FVIIa; D015942:Factor VIIa