Gray platelet syndrome
Other Names for this Disease
- Marked decrease or absence of alpha-granules and of platelet-specific alpha-granule proteins
- Platelet alpha-granule deficiency
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Signs and symptoms usually appear at birth or in early childhood and include low platelet counts, easy bruising, prolonged bleeding, and nose bleeds. Affected individuals often have myelofibrosis and splenomegaly. Bleeding tendency is usually mild to moderate in those with mild thrombocytopenia. However, the thrombocytopenia and myelofibrosis are usually progressive in nature. GPS may result in fatal hemorrhage (bleeding), especially in adulthood when platelet counts are further decreased. Female patients may develop heavy menstrual bleeding.
Last updated: 10/3/2011
- Gunay-Aygun M & Gahl WA. Gray platelet syndrome. Orphanet. June 2011; http://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=721. Accessed 10/3/2011.