Gray platelet syndrome

Gray platelet syndrome
Other namesPlatelet alpha-granule deficiency
Gray platelet syndrome is inherited in an autosomal recessive manner.
SpecialtyHematology 

Gray platelet syndrome (GPS), or platelet alpha-granule deficiency,[1] is a rare congenital autosomal recessive bleeding disorder caused by a reduction or absence of alpha-granules in blood platelets, and the release of proteins normally contained in these granules into the marrow, causing myelofibrosis.[2] The name derives from the initial observation of gray appearance of platelets with a paucity of granules on blood films from a patient with a lifelong bleeding disorder.[3]

  1. ^ Online Mendelian Inheritance in Man (OMIM): 139090
  2. ^ Nurden AT, Nurden P (January 2007). "The gray platelet syndrome: clinical spectrum of the disease". Blood Reviews. 21 (1): 21–36. doi:10.1016/j.blre.2005.12.003. PMID 16442192.
  3. ^ Rao, A. Koneti; Rao, Deepak A. (2020-10-22). "Gray platelet syndrome: immunity goes awry". Blood. 136 (17): 1898–1900. doi:10.1182/blood.2020008196. ISSN 0006-4971. PMC 7582557. PMID 33091137.
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