Artículos de revistas
Diagnosis of Scott syndrome in patient with bleeding disorder of unknown cause
Registro en:
Blood Coagulation & Fibrinolysis. Lippincott Williams & Wilkins, v. 23, n. 1, n. 75, n. 77, 2012.
0957-5235
WOS:000298627400013
10.1097/MBC.0b013e32834d0c81
Autor
Flores-Nascimento, MC
Orsi, FLA
Yokoyama, AP
Pereira, FG
Lorand-Metze, I
De Paula, EV
Castro, V
Annichino-Bizzacchi, JM
Institución
Resumen
Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP) Scott syndrome is a rare bleeding disorder due to an impaired exposure of phosphatidilserine on the platelet membrane, compromising the platelet procoagulant activity, thrombin generation and, thus, the clot formation. We report a case of a 17-year-old female adolescent with bleeding episodes of unknown cause. She had normal coagulation, but altered platelet aggregation under arteriolar flow, indicating platelet dysfunction. Furthermore, the expression of Annexin V was markedly reduced and the diagnosis of Scott syndrome was established. She was treated with platelet transfusions and demonstrated a clinical improvement. Scott syndrome may be investigated in cases with bleeding history and normal coagulation tests. Blood Coagul Fibrinolysis 23:75-77 (C) 2011 Wolters Kluwer Health vertical bar Lippincott Williams & Wilkins. 23 1 75 77 Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP) Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP)