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Identification of a new congenital defect of platelet function
characterized by severe impairment of platelet responses to adenosine
diphosphate
M Cattaneo, A Lecchi, AM Randi, JL McGregor and PM Mannucci
A. Bianchi Bonomi Hemophilia and Thrombosis Center, IRCCS Maggiore
Hospital, Milan, Italy.
This study characterizes a congenital hemorrhagic disorder caused by a
platelet function defect with the following features: (1) severely impaired
platelet aggregation and fibrinogen or von Willebrand factor (vWF) binding
induced by adenosine diphosphate (ADP); (2) defective aggregation, release
reaction, and fibrinogen or vWF binding induced by other agonists; (3)
normal aggregation and release reaction induced by high concentrations of
thrombin or collagen; (4) no further inhibition by ADP scavengers of
aggregation, release reaction, and fibrinogen or vWF binding, comparable
with those observed for normal platelets in the presence of ADP scavengers;
(5) normal membrane glycoprotein (GP) composition and normal binding of the
anti-GP IIb/IIIa monoclonal antibody 10E5; (6) no acceleration by ADP of
binding of the anti-GP IIb/IIIa monoclonal antibody 7E3; (7) normal
platelet-fibrin clot retraction if induced by thrombin or reptilase plus
epinephrine, absent if induced by reptilase plus ADP; (8) no inhibition by
ADP of the prostaglandin E1-induced increase in platelet cyclic adenosine
monophosphate, but normal inhibition by epinephrine; (9) defective
mobilization of cytoplasmic Ca2+ by ADP; (10) normal binding of 14C-ADP to
fresh platelets, but defective binding of [2-3H]-ADP to formalin- fixed
platelets. This congenital platelet function defect is characterized by
selective impairment of platelet responses to ADP, caused by either
decreased number of platelet ADP receptors or abnormalities of the
signal-transduction pathway of platelet activation by ADP.
Volume 80,
Issue 11,
pp. 2787-2796,
12/01/1992
Copyright © 1992 by The American Society of Hematology

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