Gray 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
References in periodicals archive ?
Platelet alpha granule deficiency associated with decreased P-selectin and selective impairment of thrombin-induced activation in a new patient with the gray platelet syndrome ([alpha]-storage pool deficiency).