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Related Experiment Videos

Circulating activated endothelial cells in sickle cell anemia

A Solovey1, Y Lin, P Browne

  • 1Department of Medicine, University of Minnesota Medical School, Minneapolis, USA.

The New England Journal of Medicine
|November 27, 1997
PubMed
Summary

Endothelial cells are activated in sickle cell anemia, regardless of clinical status. These activated cells, expressing adhesion molecules, may play a role in the vascular pathology of the disease.

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Area of Science:

  • Hematology
  • Vascular Biology
  • Pathophysiology

Background:

  • The vascular wall is implicated in sickle cell disease pathogenesis.
  • Endothelial activation in sickle cell disease requires further investigation.

Purpose of the Study:

  • To determine if the endothelium is activated in sickle cell anemia.
  • To study the number, origin, and surface phenotype of circulating endothelial cells in patients with sickle cell anemia.

Main Methods:

  • Immunohistochemical examination of buffy-coat smears to enumerate circulating endothelial cells.
  • Evaluation of surface phenotype using immunofluorescence microscopy with a panel of antibodies, including anti-endothelial-cell antibody P1H12.

Main Results:

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  • Patients with sickle cell anemia showed significantly higher numbers of circulating endothelial cells (22.8/mL during acute episodes, 13.2/mL otherwise) compared to healthy donors (2.6/mL).
  • Circulating endothelial cells in sickle cell anemia patients were predominantly microvascular in origin and expressed markers of endothelial activation (ICAM-1, VCAM-1, E-selectin, P-selectin).
  • Higher levels of circulating endothelial cells tended to occur at the onset of acute painful crises.

    • Conclusions:

    • The vascular endothelium is activated in sickle cell anemia patients, irrespective of their clinical status.
    • Adhesion proteins on activated endothelial cells may contribute to the vascular pathology observed in sickle cell disease.