The Endothelial Biology of Sickle Cell Disease: Inflammation and a Chronic Vasculopathy

  1. ROBERT P. HEBBEL1,*,
  2. RAYMOND OSAROGIAGBON2,
  3. DHANANJAY KAUL3

Article first published online: 26 JAN 2010

DOI: 10.1080/10739680490278402

Issue

Microcirculation

Microcirculation

Volume 11, Issue 2, pages 129–151, March 2004

Additional Information

How to Cite

HEBBEL, R. P., OSAROGIAGBON, R. and KAUL, D. (2004), The Endothelial Biology of Sickle Cell Disease: Inflammation and a Chronic Vasculopathy. Microcirculation, 11: 129–151. doi: 10.1080/10739680490278402

Author Information

  1. 1

    Vascular Biology Center and Division of Hematology–Oncology–Transplantation, Department of Medicine, University of Minnesota Medical School, Minneapolis, Minnesota, USA

  2. 2

    Department of Medicine, Veterans Administration Center, Amarillo, Texas, USA

  3. 3

    Division of Hematology, Department of Medicine, Albert Einstein College of Medicine, Bronx, New York, USA

*Address correspondence to Dr. Robert P. Hebbel, Department of Medicine, University of Minnesota Medical School, MMC 480, 420 Delaware St. SE, Minneapolis, MN 55455, USA. E-mail: hebbe001@umn.edu.

Publication History

  1. Issue published online: 26 JAN 2010
  2. Article first published online: 26 JAN 2010
  3. Received 9 June 2003; accepted 9 June 2003.

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Keywords:

  • endothelium;
  • inflammation;
  • sickle;
  • vascular

ABSTRACT

A single amino acid substitution in hemoglobin comprises the molecular basis for sickle cell anemia, but evolution of the corresponding clinical disease is extraordinarily complicated and likely involves multiple pathogenic factors. Sickle disease is fundamentally an inflammatory state, with activation of the endothelium, probably through proximate effects of reperfusion injury physiology and chronic molestation by adherent red cells and white cells. The disease also involves enhanced angiogenic propensity, activation of coagulation, disordered vasoregulation, and a component of chronic vasculopathy. Sickle cell anemia is truly an endothelial disease, and it is likely that genetic differences in endothelial function help govern its astonishing phenotypic diversity.

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