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 Blood, 1 July 2004, Vol. 104, No. 1, pp. 107-114.
Prepublished online as a Blood First Edition Paper on March 9, 2004; DOI 10.1182/blood-2003-11-3842.

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HEMOSTASIS, THROMBOSIS, AND VASCULAR BIOLOGY

Severe deficiency of glycoprotein VI in a patient with gray platelet syndrome

Paquita Nurden, Martine Jandrot-Perrus, Robert Combrié, Joelle Winckler, Veronique Arocas, Christelle Lecut, Jean-Max Pasquet, Thomas J. Kunicki, and Alan T. Nurden

From IFR4, Laboratoire d'Hématologie, Hôpital Cardiologique, Pessac, France; E348 Institut National de la Santé et de la Recherche Médicale (INSERM), Faculté Xavier Bichat, Paris, France; FRE 2617 Centre National de la Recherche Scientifique (CNRS), Université de Bordeaux2, Bordeaux, France; and The Roon Research Center for Arteriosclerosis and Thrombosis, The Scripps Research Institute, La Jolla, CA.

We report a novel case of gray platelet syndrome (GPS) where a severe deficiency of the platelet collagen receptor, glycoprotein (GP) VI, accompanies classical symptoms of a low platelet count and platelets lacking {alpha}-granules. Dense granules were normally present. Platelet aggregation with collagen was severely decreased, as was the response to convulxin (Cvx), a GPVI agonist. Quantitative analysis of GPVI using fluorescein isothiocyanate (FITC)–Cvx in flow cytometry showed its virtual absence on the patient's platelets. The GPVI deficiency was confirmed using monoclonal antibodies in Western blotting and in immunogold labeling on frozen thin sections where internal pools of GPVI were confirmed for normal platelets. The Fc receptor {gamma}-chain, constitutively associated with GPVI in normal platelets, was present in subnormal amounts, and the phospholipase C{gamma}2–dependent activation pathway appeared to function normally. No autoantibodies to GPVI were found in the patient's serum using monoclonal antibody immobilization of platelet antigen (MAIPA). Sequencing of coding regions of the GPVI gene failed to show abnormalities, and mRNA for GPVI was present in the patient's platelets, pointing to a probable acquired defect in GPVI expression. Our results may provide a molecular explanation for the subgroup of patients with severely deficient collagen-induced platelet aggregation as previously described for GPS in the literature.


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