EVIDENCE THAT ABNORMAL PLATELET FUNCTIONS IN HUMAN CHEDIAK-HIGASHI-SYNDROME ARE THE RESULT OF A LACK OF DENSE BODIES

  • 1 January 1983
    • journal article
    • research article
    • Vol. 111  (3) , 307-314
Abstract
The structure and functions of platelets from 3 patients with the Chediak-Higashi syndrome were examined. Electron-microscopic observations revealed a large reduction in the number of serotonin-storage granules or dense bodies but otherwise normal ultrastructure and normal amounts of .alpha.-granules and catalase-positive granules. The number of mepacrine-labeled granules was also reduced. Platelets contained normal amounts of .beta.-thromboglobulin and Platelet Factor 4. The platelet release reaction studied with thrombin as the inducer was impaired. The serotonin uptake by the patients'' platelets was low and not inhibited by reserpine, and its metabolism was increased. Apparently, platelets from human Chediak-Higashi syndrome are deficient in the storage pool of dense granule substances, and this granule defect evidently has an influence on the release mechanism of other granule constituents.