grey platelet syndrome


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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

grey platelet syndrome

A genetic disorder of platelet granule secretion in which the platelets, which are reduced in number, show a distinctive grey appearance with Romanowsky stains and have few or no granules. The condition features a mild bleeding tendency. This is one of the range of inherited throbocytopenias.