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.
KEYWORDS
Hypercoagulability - vascular endothelial dysfunction - inflammation - neutrophils
- dense red cells
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Lucia De FranceschiM.D.
Department of Medicine, University of Verona, Policlinico GB Res8i
P.le L Scuro, 10, 37134 Verona, Italy
eMail: lucia.defranceschi@univr.it
