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, A Pecci, G Ciabattoni, Raimondo De Cristofaro, Sergio Rutella, Z Yao Wu, Ilaria Lazzareschi, Raffaele Landolfi, S Coughlin, Cl Balduini

Risultato della ricerca: Contributo in rivistaArticolo

24 Citazioni (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.
Lingua originaleInglese
pagine (da-a)551-559
Numero di pagine9
RivistaJournal of Thrombosis and Haemostasis
Volume5
Numero di pubblicazione3
DOI
Stato di pubblicazionePubblicato - 2007

All Science Journal Classification (ASJC) codes

  • Ematologia

Keywords

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

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