Neutrophils in the gray platelet syndrome

Abstract

A recent report of three siblings with the gray platelet syndrome (GPS) and two others, a brother and sister, has indicated that patients with the GPS have gray neutrophils, as well as gray platelets. Their neutrophils are markedly deficient in secondary granules and vesicles and the empty cytoplasm appears gray on peripheral blood smears. We have evaluated five patients with the GPS, including the original case described by Raccuglia (Am J Med 1971; 51: 818–28). The results indicate that GPS neutrophils have normal secondary granules, and they are not gray on peripheral blood smears or in thin sections. The patients described in the recent report by the French workers appear to have a familial hypogranular neutrophil and platelet disorder, but it does not appear to be the GPS.