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Blood Coagul Fibrinolysis. 2012 Jan;23(1):75-7. doi: 10.1097/MBC.0b013e32834d0c81.

Diagnosis of Scott syndrome in patient with bleeding disorder of unknown cause.

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  • 1Hematology and Hemotherapy Center, State University of Campinas (UNICAMP), Campinas, Sao Paulo, Brazil. flores_mariane@yahoo.com.br

Abstract

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.

PMID:
22027759
DOI:
10.1097/MBC.0b013e32834d0c81
[PubMed - indexed for MEDLINE]
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