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
