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Hemophilia Care in the New Millennium pp 13–29Cite as

Are Patients with Glanzmann Thrombasthenia and the Bernard-Soulier Syndrome Protected Against Atherosclerosis?

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Part of the book series: Advances in Experimental Medicine and Biology ((AEMB,volume 489))

Abstract

Genetic defects of platelets give rise to bleeding syndromes of varying severity. Affected areas of platelet function include: the glycoprotein (GP) effectors of adhesion and aggregation, primary receptors for agonists, signalling pathways whereby messages are transmitted to targets elsewhere in the membrane or within the platelet, secretion from dense and a-granules, and the expression of procoagulant activity. Glanzmann thrombasthenia (GT) and the Bernard-Soulier syndrome (BSS) are the best characterized platelet diseases (1,2). GT is caused by abnormalities of platelet membrane GP IIb-IIIa (integrin αIIBβ;3), resulting in absent platelet aggregation. BSS is caused by abnormalities of the GP Ib-V-IX complex, resulting in a loss of platelet adherence to vessel wall subendothelium. The knowledge gained from studies on platelet disorders has provided a better understanding of the molecular basis of primary hemostasis, and has helped in the development of new drugs for use in antithrombotic therapy. At the same time, the inability of platelets to bind to injured sites within the wall or to aggregate and form a thrombus in response to a haemostatic challenge raises the question as to whether such patients will be protected against atherosclerosis, for among the consequences of the altered platelet reactivity will be a decreased release of cytokines and growth factors potentially involved in atherosclerotic plaque development.

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Author information

Authors and Affiliations

  1. Laboratoire de Pathologie Cellulaire de l’Hémostase UMR 5533 CNRS, Hôpital Cardiologique, Pessac, France

    Alan T. Nurden & Paquita Nurden

  2. Department of Medicine, Hematology-Oncology Section, University of Oklahoma Health Sciences Center, Oklahoma City, OK

    James N. George

Authors
  1. Alan T. Nurden
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  2. Paquita Nurden
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  3. James N. George
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Editor information

Editors and Affiliations

  1. University of North Carolina School of Medicine, Chapel Hill, North Carolina, USA

    Dougald M. Monroe

  2. Novo Nordisk A/S, Målov, Denmark

    Ulla Hedner

  3. University of Lund, Lund, Sweden

    Ulla Hedner

  4. Duke University and Durham VA Medical Centers, Durham, North Carolina, USA

    Maureane R. Hoffman

  5. Hôpital Edouard Herriot, Lyon, France

    Claude Negrier

  6. Guys’ and St. Thomas’s Trust, London, UK

    Geoffrey F. Savidge

  7. University of North Carolina School of Medicine, Chapel Hill, North Carolina, USA

    Gilbert C. White II

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Nurden, A.T., Nurden, P., George, J.N. (2001). Are Patients with Glanzmann Thrombasthenia and the Bernard-Soulier Syndrome Protected Against Atherosclerosis?. In: Monroe, D.M., Hedner, U., Hoffman, M.R., Negrier, C., Savidge, G.F., White, G.C. (eds) Hemophilia Care in the New Millennium. Advances in Experimental Medicine and Biology, vol 489. Springer, Boston, MA. https://doi.org/10.1007/978-1-4615-1277-6_2

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  • DOI: https://doi.org/10.1007/978-1-4615-1277-6_2

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