Open AccessDiagnosis of Scott syndrome in patient with bleeding disorder of unknown cause
Author(s) -
Mariane Cristina Flores-Nascimento,
Fernanda Andrade Orsi,
Ana Paula Hitomi Yokoyama,
Fernanda Pereira,
Irene Lorand-Metze,
Erich Vinícius De Paula,
Vagner Castro,
Joyce Maria AnnichinoBizzacchi
Publication year - 2012
Publication title -
blood coagulation and fibrinolysis
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.376
H-Index - 71
eISSN - 1473-5733
pISSN - 0957-5235
DOI - 10.1097/mbc.0b013e32834d0c81
Subject(s) - platelet , medicine , coagulation , platelet activation , coagulation disorder , annexin , coagulation testing , gastroenterology , cardiology , surgery , pathology , immunology , flow cytometry
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.