The endothelial biology of sickle cell disease: Inflammation and a chronic vasculopathy

Robert P. Hebbel, Raymond Osarogiagbon, Dhananjay Kaul

Research output: Contribution to journalReview articlepeer-review

322 Scopus citations

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.

Original languageEnglish (US)
Pages (from-to)129-151
Number of pages23
JournalMicrocirculation
Volume11
Issue number2
DOIs
StatePublished - Mar 2004

Keywords

  • Endothelium
  • Inflammation
  • Sickle
  • Vascular

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