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Gray platelet syndrome

Disease definition

Gray platelet syndrome (GPS) is a rare inherited bleeding disorder characterized by macrothrombocytopenia, myelofibrosis, splenomegaly and typical gray appearance of platelets on Wright stained peripheral blood smear.

ORPHA:721

  • Synonym(s):
    • Alpha storage pool deficiency
    • GPS
    • Platelet alpha-granule deficiency
  • Prevalence: <1 / 1 000 000
  • Inheritance: Autosomal dominant or Autosomal recessive 
  • Age of onset: Childhood
  • ICD-10: D69.1
  • OMIM: 139090  187900
  • UMLS: C0272302
  • MeSH: D055652
  • GARD: 2562
  • MedDRA: -

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