PLATELET-FUNCTION IN CHEDIAK-HIGASHI-SYNDROME

  • 1 January 1976
    • journal article
    • research article
    • Vol. 47  (6) , 941-948
Abstract
Platelet function studies were performed on 2 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, and 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 an explanation for impaired primary hemostasis in these patients and illustrate another disorder in which platelet storage-pool deficiency occurs.