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Related Experiment Videos

Thrombogenesis in sickle cell disease.

A Tomer1, L A Harker, S Kasey

  • 1Department of Hematology and Oncology, Winship Cancer Institute, Emory University School of Medicine, Atlanta, GA 30303, USA.

The Journal of Laboratory and Clinical Medicine
|June 1, 2001
PubMed
Summary

Sickle cell disease (SCD) patients show increased platelet activation and blood clotting markers, even when pain-free. These thrombogenic activities intensify during pain episodes, predicting future pain frequency and interval.

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Area of Science:

  • Hematology
  • Vascular Biology
  • Thrombosis

Background:

  • Sickle cell disease (SCD) is characterized by vaso-occlusion and pain crises.
  • The role of thrombogenic activity in SCD pathophysiology requires further elucidation.

Purpose of the Study:

  • To investigate in vivo thrombogenic activities in SCD patients.
  • To compare these activities between pain episodes and pain-free periods.
  • To assess the relationship between thrombogenic markers and pain frequency.

Main Methods:

  • Flow cytometry was used to assess platelet activation (GPIIb/IIIa, P-selectin) and procoagulant activity (FVa, annexin V binding) on platelets and erythrocytes.
  • Plasma levels of platelet factors (PF4, betaTG), thrombin generation markers (F(1.2), TAT), and fibrinolysis markers (D-dimer, PAP) were measured.

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  • Correlations between pain frequency and thrombogenic markers were analyzed.

    • Main Results:

    • Asymptomatic SCD patients exhibited elevated platelet activation, PF4, betaTG, thrombin generation, and fibrinolysis compared to controls.
    • During pain episodes, platelet activation, erythrocyte procoagulant activity, and platelet microparticles significantly increased.
    • SCD pain episodes were associated with higher levels of thrombin generation and fibrinolysis markers.
    • Pain frequency correlated with enhanced platelet procoagulant activity and fibrinolysis during pain-free periods.

    Conclusions:

    • SCD patients exhibit persistent thrombogenic activity, including platelet activation, thrombin generation, and fibrinolysis.
    • These activities escalate during pain episodes, suggesting a role in SCD pathophysiology.
    • Thrombogenic markers may predict pain frequency and recurrence in SCD.