Gray platelet syndrome
Gray platelet syndrome (GPS) is a rare inherited condition that affects platelets, whose irregular shape and sticky surface allow them to help form clots to stop blood loss on the inside and outside of the body. In GPS, the platelets do not function properly, which interferes with the process of clotting and may lead to prolonged bleeding time, excessive blood loss, and anemia.
GPS is caused by decreased or absent alpha-granules, a type of granule, or vesicle, that contains several types of growth factors, such as insulin growth factor, platelet-derived growth factor, platelet factor 4, and other clotting proteins. The platelets in patients with GPS tend to be large and contain few granules, which gives the cells a gray color. Researchers believe that GPS is caused by a genetic mutation or defect, although the specific location of the mutation is currently unknown.
There is limited information regarding the prevalence of GPS. The condition appears to affect males, females, and individuals of all ethnic and racial groups in equal numbers. The life expectancy of patients with GPS is unknown.
Alpha-granule deficiency, desmopressin, GPS, gray platelet syndrome, platelet alpha-granule deficiency, platelet transfusion, thrombocytopenia.