Thrombosis and sickle cell disease

Semin Thromb Hemost. 2011 Apr;37(3):226-36. doi: 10.1055/s-0031-1273087. Epub 2011 Mar 31.


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.

Publication types

  • Review

MeSH terms

  • Anemia, Sickle Cell / blood*
  • Anemia, Sickle Cell / drug therapy
  • Anticoagulants / therapeutic use
  • Blood Coagulation / physiology
  • Blood Platelets / physiology
  • Endothelium, Vascular / physiopathology
  • Erythrocytes / metabolism
  • Erythrocytes / pathology
  • Erythrocytes, Abnormal / pathology
  • Hemoglobin, Sickle / metabolism
  • Humans
  • Inflammation / physiopathology
  • Leukocytes / physiology
  • Nitric Oxide / metabolism
  • Platelet Activation / genetics
  • Platelet Aggregation Inhibitors / therapeutic use


  • Anticoagulants
  • Hemoglobin, Sickle
  • Platelet Aggregation Inhibitors
  • Nitric Oxide