Semin Thromb Hemost 2011; 37(3): 226-236
DOI: 10.1055/s-0031-1273087
© Thieme Medical Publishers

Thrombosis and Sickle Cell Disease

Lucia De Franceschi1 , Maria Domenica Cappellini2 , Oliviero Olivieri1
  • 1Department of Medicine, University of Verona, Verona, Italy
  • 2Policlinico Foundation, Istituto di Ricovero e Cura a Carattere Scientifico (IRCCS), University of Milan, Milan, Italy
Further Information

Publication History

Publication Date:
31 March 2011 (online)


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.


Lucia De FranceschiM.D. 

Department of Medicine, University of Verona, Policlinico GB Res8i

P.le L Scuro, 10, 37134 Verona, Italy