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American Journal of Pathology, Vol 111, 307-314, Copyright © 1983 by American Society for Investigative Pathology

REGULAR ARTICLES

Evidence that abnormal platelet functions in human Chediak-Higashi syndrome are the result of a lack of dense bodies

F Rendu, J Breton-Gorius, M Lebret, C Klebanoff, D Buriot, C Griscelli, S Levy- Toledano and JP Caen

The structure and functions of platelets from three 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. These findings clearly show that platelets from human Chediak-Higashi syndrome are deficient in the storage pool of dense granule substances and suggest that this granule defect has an influence on the release mechanism of other granule constituents.


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Copyright © 1983 by the American Society for Investigative Pathology.