Hereditary giant platelet syndrome: a disorder of a new aspect of platelet function.
The platelets of three patients with the hereditary giant platelet syndrome of Bernard and Soulier failed to aggregate in response to either ristocetin or bovine fibrinogen. The results of aggregation experiments using mixtures of platelets and plasma suggest that a reaction between a plasma factor deficient in von Willebrand's disease and a platelet component lacking in our patients, and leading to platelet aggregation independently of adenosine diphosphate (ADP), is essential for normal haemostasis.
D000244:Adenosine Diphosphate / Q000494:pharmacology; D000293:Adolescent; D001791:Blood Platelet Disorders / Q000235:genetics*; D002648:Child; D003094:Collagen / Q000494:pharmacology; D005260:Female; D005340:Fibrinogen / Q000494:pharmacology; D006470:Hemorrhage; D006801:Humans; D010949:Plasma; D010973:Platelet Adhesiveness / Q000187:drug effects; D011516:Prothrombin / Q000378:metabolism; D011696:Purpura, Thrombocytopenic / Q000235:genetics*; D012310:Ristocetin / Q000494:pharmacology; D013577:Syndrome; D013917:Thrombin / Q000494:pharmacology; D014842:von Willebrand Diseases / Q000378:metabolism
D012310:Ristocetin; D000244:Adenosine Diphosphate; D011516:Prothrombin; D005340:Fibrinogen; D003094:Collagen; D013917:Thrombin