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Deficiency of platelet membrane glycoprotein Ia associated with a decreased
platelet adhesion to subendothelium: a defect in platelet spreading
HK Nieuwenhuis, KS Sakariassen, WP Houdijk, PF Nievelstein and JJ Sixma
A bleeding disorder with absent collagen-induced platelet aggregation and
adhesion has been described in a patient whose platelets failed to express
surface glycoprotein Ia. We studied the interaction of her platelets with
subendothelium in an annular perfusion chamber and the interaction with
purified human collagen type III in a rectangular perfusion system under
flow conditions. Platelet adherence was almost completely absent both at
low and high shear rates. The few platelets which adhered remained in the
contact stage without subsequent spreading and aggregate formation.
Addition of a monoclonal antibody, which was directed against the von
Willebrand moiety of FVIII-VWF, to the blood, completely abolished platelet
adherence at high shear rates and had a partial effect at low shear rates.
These data indicate that von Willebrand factor plays a role in the initial
attachment (contact stage) of platelets to subendothelium. We conclude that
the bleeding disorder and excessively prolonged bleeding time in our
patient are caused by a new specific defect of the platelet-vessel wall
interaction.
Volume 68,
Issue 3,
pp. 692-695,
09/01/1986
Copyright © 1986 by The American Society of Hematology
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