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).
a) Abnormalities of adhesion (platelet vessel wall interaction/adhesion) VWD Bernard Soulier syndrome (abnormal or absent GP Ib) Abnormalities of platelet aggregation Congenital afibrinogenemia Glanzmann thrombasthenia (abnormal GP Ilb/IIIa) Disorders of platelet release/signal transduction Storage pool disease Deficient dense bodies Hermansky-Pudlak syndrome Chediak-Higashi syndrome Wiskott-Aldrich syndrome Thrombocytopenia with absent radii Deficient [alpha] granules Gray platelet syndrome Deficiency of [alpha] granules and dense bodies Signal transduction defects Abnormal AA pathways Impaired AA release Cyclooxygenase deficiency Thromboxane synthetase deficiency Abnormalities of platelet membrane response Scott syndrome (a) From Rao (2).