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

Erica De Candia, Raimondo De Cristofaro, Ilaria Lazzareschi, Sergio Rutella, Raffaele Landolfi, A. Pecci, G. Ciabattoni, Z. Yao-Wu, S. Coughlin, C. L. Balduini

Research output: Contribution to journalArticle

24 Citations (Scopus)

Abstract

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.
Original languageEnglish
Pages (from-to)551-559
Number of pages9
JournalJournal of Thrombosis and Haemostasis
Volume5
DOIs
Publication statusPublished - 2007

Keywords

  • Adolescent
  • Adult
  • Aged
  • Blood Platelets
  • Calcium Signaling
  • Coagulants
  • Cytoplasmic Granules
  • Family
  • Female
  • Humans
  • Male
  • Microscopy, Fluorescence
  • Middle Aged
  • Oligopeptides
  • P-Selectin
  • Pedigree
  • Phenotype
  • Platelet Aggregation
  • Platelet Factor 4
  • Platelet Function Tests
  • Platelet Storage Pool Deficiency
  • Receptor, PAR-1
  • Syndrome
  • Thrombin
  • Thrombospondin 1
  • Thromboxane B2

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