Thrombosis and sickle cell disease

Lucia De Franceschi, Maria Domenica Cappellini, Oliviero Olivieri

Research output: Contribution to journalArticle

Abstract

Sickle cell disease (SCD) is characterized by the presence of sickle hemoglobin, which has the unique property of polymerizing when deoxygenated. The pathophysiology of acute and chronic clinical manifestations of SCD have shown the central role of dense, dehydrated red cells in acute and chronic clinical manifestations of this pathology. Recent studies have indicated that SCD is characterized by a hypercoagulable state that contributes to the vaso-occlusive events in microcirculation, leading to acute and chronic sickle cell-related organ damage. This review discusses, in the context of SCD, (1) abnormalities in the coagulation system, (2) perturbation of platelet activation and aggregation, (3) vascular endothelial dysfunction, (4) the contribution of cell inflammatory responses, and (5) the connection with nitric oxide metabolism. We also review the available studies on the therapeutic approaches in clinical management of hypercoagulability in SCD.

Original languageEnglish
Pages (from-to)226-236
Number of pages11
JournalSeminars in Thrombosis and Hemostasis
Volume37
Issue number3
DOIs
Publication statusPublished - 2011

    Fingerprint

Keywords

  • dense red cells
  • Hypercoagulability
  • inflammation
  • neutrophils
  • vascular endothelial dysfunction

ASJC Scopus subject areas

  • Hematology
  • Cardiology and Cardiovascular Medicine

Cite this