TY - JOUR
T1 - Platelet function in the Chediak Higashi syndrome
AU - Buchanan, G. R.
AU - Handin, R. I.
N1 - Copyright:
Copyright 2020 Elsevier B.V., All rights reserved.
PY - 1976
Y1 - 1976
N2 - 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, 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 an explanation for impaired primary hemostasis in these patients, and illustrate another disorder in which platelet storage pool deficiency occurs.
AB - 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, 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 an explanation for impaired primary hemostasis in these patients, and illustrate another disorder in which platelet storage pool deficiency occurs.
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U2 - 10.1182/blood.v47.6.941.bloodjournal476941
DO - 10.1182/blood.v47.6.941.bloodjournal476941
M3 - Article
C2 - 1276475
AN - SCOPUS:0017136389
SN - 0006-4971
VL - 47
SP - 941
EP - 948
JO - Blood
JF - Blood
IS - 6
ER -