Gray Platelet Syndrome | definition of Gray Platelet Syndrome by Medical dictionary
Gray Platelet Syndrome
Also found in: Acronyms
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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References in periodicals archive
In addition, [alpha]-SPD (Gray platelet syndrome
) has decreased alpha granules and is usually considered a macrothrombocytopenia.
Ultrastructural ab-normalities in 8-SPD show absent to decreased dense granules.' The alpha storage pool disorder 8-SPD leads to gray platelet syndrome
(GPS) with large (mean 13 fL) platelets devoid of alpha granules, giving them a ghostly gray color on the peripheral smear.
Patients with [Alpha]-SPD were originally described as having a "gray platelet syndrome
" because of the agranular appearance of their platelets on peripheral blood smears.