Hereditary giant platelet syndrome. Absence of collagen-induced coagulant activity and deficiency of factor-XI binding to platelets.

British Journal of Haematology
P N WalshJ J Egan

Abstract

The platelets from two related patients with the hereditary giant platelet syndrome were examined. They were larger than normal but otherwise ultrastructurally normal; they contained increased storage pools of adenine nucleotides and heparin-neutralizing activity and took up serotonin at an increased rate. They aggregated normally with ADP and collagen but failed to aggregate with bovine factor VIII and Ristocetin. Some change in shape occurred with ADP, and the reduction in adenylate energy change after addition of ADP to platelet-rich plasma was smaller than normal. Platelet coagulant activities including contact product forming activity, intrinsic factor-Xa forming activity and platelet factor 3 activity were normal or increased, but collagen-induced coagulant activity was absent whether tested in washed platelet suspensions or platelet-rich plasma. Platelet washing experiments showed decreased binding of factors V and VIII to hereditary giant platelets and no detectable factor XI in washed platelet suspensions. It is concluded that (1) the hereditary giant platelets studied lacked a binding mechanism for factors, V, VIII and XI; (2) the normal development of collagen-induced coagulant activity apparently depends upon the bi...Continue Reading

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Citations

May 1, 1976·British Journal of Haematology·P N Walsh, M S Lipscomb
Jan 29, 2005·Clinical and Applied Thrombosis/hemostasis : Official Journal of the International Academy of Clinical and Applied Thrombosis/Hemostasis·Anna M Dyszkiewicz-KorpantyRavindra Sarode
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Aug 8, 2009·Arteriosclerosis, Thrombosis, and Vascular Biology·Tara C White-AdamsOwen J T McCarty
Feb 22, 2013·British Journal of Pharmacology·Joanna LeckaJean Sévigny
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Jul 1, 2015·Blood Coagulation & Fibrinolysis : an International Journal in Haemostasis and Thrombosis·Manasa Krishnegowda, Vani Rajashekaraiah
Aug 1, 1979·The American Journal of Medicine·H J WeissJ Rogers

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