Article
Defective platelet responsiveness to thrombin and protease-activated receptors agonists in a novel case of gray platelet syndrome: correlation between the platelet defect and the alpha-granule content in the patient and four relatives.
Journal of thrombosis and haemostasis : JTH - 1 Mar 2007
De Candia E, Pecci A, Ciabattoni G, De Cristofaro R, Rutella S, Yao-Wu Z, Lazzareschi I, Landolfi R, Coughlin S, Balduini C L
Abstract excerpt
BACKGROUND: We report a novel case of gray platelet syndrome (GPS). A 14-year-old boy had bleeding diathesis, mild thrombocytopenia, giant platelets with severe defect of alpha-granule secretory proteins, myelofibrosis and splenomegaly. METHODS AND RESULTS: Platelet function studies showed a marked reduction of aggregation and Ca(2+) mobilization by thrombin, protease-activated receptor 1 (PAR1)-activating...
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