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Gray Platelet Syndrome

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Gray Platelet Syndrome
An autosomal dominant [MIM 139090] condition in which the platelets lack alpha and dense granules and by extension, certain platelet proteins—e.g., von Willebrand factor, fibrinogen, fibrin, fibronectin, platelet factor 4 (PF4), beta-thromboglobulin, PDGF, thrombospondin, and contact-promoting proteins
Lab Thrombocytopenia, enlarged platelets with a gray hue on Wright-Giemsa stained peripheral blood smears, increased bleeding time
Management DDAVP—desmopressin acetate


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The differential diagnosis of inherited giant platelet disorder with minimal to absent bleeding symptoms includes heterozygous BSS and MYH9 disorders, gray platelet syndrome, and HPS.
94) The Gray platelet syndrome is an autosomal dominant [alpha]-SPD characterized by mild bleeding symptoms, reticulin fibrosis of the bone marrow, variable thrombocytopenia, and large (mean, 13 fL), gray-appearing platelets on the peripheral blood smear due to decreased alpha granules.
 
 
 
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