Gray platelet syndrome | |
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Other names | Platelet alpha-granule deficiency |
Gray platelet syndrome is inherited in an autosomal recessive manner. | |
Specialty | Hematology |
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]