title: [Gray platelet syndrome].
abstract: BACKGROUND
  Gray platelet syndrome is a rare (about 40 cases published), inherited disorder characterized by a marked decrease or absence of platelet alpha-granules and platelet specific alpha-granule proteins.
  
  
  METHODS
  A boy, aged 4 years, presented with frequent ecchymoses. Acute idiopathic thrombocytopenia purpura was diagnosed because of his reduced platelet count (36,000/mm3) and recent viral infection. Intravenous gammaglobulin infusion was followed by a small rise in the platelet count (125,000/mm3). The patient was reinvestigated a few months later because of persistent thrombocytopenia and the failure of the immunologic treatment. The bleeding time was long and the platelets on blood smears appeared gray. Electron microscopy revealed numerous vacuoles and very few or no alpha-granules. Platelet aggregation and adhesion were normal, but stimulated platelets failed to liberate factor 4 and beta-thromboglobulin, while the plasma levels of beta-thromboglobulin were elevated.
  
  
  CONCLUSIONS
  The frequency of gray platelet syndrome is probably underestimated in those diseases resulting in thrombocytopenia and this will continue until blood smears are thoroughly examined. Synthesis of platelet specific alpha-granule proteins seems normal; but these proteins cannot be stored as there are very few, or no alpha-granules. This abnormality could lead to increased levels of such proteins in the plasma.
authors: Service de Pédiatrie 4 (Unité d'Hémato-Oncologie Pédiatrique), INSERM U 311, Strasbourg.
authors: P
authors: P
authors: Lutz
authors: A
authors: A
authors: Roth-Pougheon
authors: M L
authors: ML
authors: Wiesel
authors: A
authors: A
authors: Albert
authors: C
authors: C
authors: Brisson
authors: L
authors: L
authors: Grunebaum
authors: J P
authors: JP
authors: Cazenave
authors: J M
authors: JM
authors: Lévy
mesh_terms: D001791:Blood Platelet Disorders / Q000150:complications* / Q000473:pathology / Q000503:physiopathology; D001792:Blood Platelets / Q000473:pathology; D002675:Child, Preschool; D003594:Cytoplasmic Granules / Q000473:pathology; D006801:Humans; D008297:Male; D016553:Purpura, Thrombocytopenic, Idiopathic / Q000150:complications*; D013577:Syndrome
vernacular_title: Le syndrome des plaquettes grises.