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Platelet function in the Chediak-Higashi syndrome

GR Buchanan and RI Handin

Platelet function studies were performed on two patients with the Chediak-Higashi syndrome, one of whom had a history of easy bruising unrelated to thrombocytopenia. Both patients had prolonged bleeding times, abnormal platelet aggregation, and a defect of platelet storage granules, manifested by reduced platelet ADP, an increased ATP/ADP ratio, increased adenine nucleotide specific radioactivity after 3H- adenine labeling, and decreased platelet uptake of radioactive 5- hydroxytryptamine. These findings confirm preliminary data in animals with the Chediak-Higashi syndrome, provide and explanation for impaired primary hemostasis in these patients, and illustrate another disorder in which platelet storage-pool deficiency occurs.

Volume 47, Issue 6, pp. 941-948, 06/01/1976
Copyright © 1976 by The American Society of Hematology


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ANN INTERN MEDHome page
S. J. SHATTIL and J. S. BENNETT
Platelets and Their Membranes in Hemostasis: Physiology and Pathophysiology
Ann Intern Med, January 1, 1981; 94(1): 108 - 118.
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  Copyright © 1976 by American Society of Hematology         Online ISSN: 1528-0020