Platelet function in the Chediak-Higashi syndrome.

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