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Related Experiment Videos

[Von Willebrand factor multimers in systemic scleroderma].

R Valsecchi, T Cainelli, E Perticucci

    Giornale Italiano Di Dermatologia E Venereologia : Organo Ufficiale, Societa Italiana Di Dermatologia E Sifilografia
    |May 1, 1989
    PubMed
    Summary
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    Patients with scleroderma have abnormal, large von Willebrand factor multimers in their plasma. These supranormal multimers may drive disease progression by promoting platelet aggregation and adhesion.

    Area of Science:

    • Vascular biology
    • Hematology
    • Immunology

    Context:

    • Endothelial cell damage is critical in scleroderma pathogenesis.
    • Scleroderma involves the formation of fibrotic lesions.

    Purpose:

    • To investigate the presence of supranormal von Willebrand factor multimers in scleroderma patients.
    • To determine if these multimers contribute to disease mechanisms.

    Summary:

    • All 11 scleroderma patients studied had supranormal von Willebrand factor multimers in their plasma.
    • These multimers were absent in healthy controls.
    • Supranormal multimers are known potent inducers of platelet aggregation and adhesion.

    Impact:

    • Findings suggest supranormal von Willebrand factor multimers may play a role in systemic sclerosis pathogenesis.

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  • This could lead to new therapeutic targets for scleroderma.