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Thrombosis and sickle cell disease.

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.

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  • Authors+Show Affiliations

    ,

    Department of Medicine, University of Verona, Verona, Italy. lucia.defranceschi@univr.it

    ,

    Source

    MeSH

    Anemia, Sickle Cell
    Anticoagulants
    Blood Coagulation
    Blood Platelets
    Endothelium, Vascular
    Erythrocytes
    Erythrocytes, Abnormal
    Hemoglobin, Sickle
    Humans
    Inflammation
    Leukocytes
    Nitric Oxide
    Platelet Activation
    Platelet Aggregation Inhibitors

    Pub Type(s)

    Journal Article
    Review

    Language

    eng

    PubMed ID

    21455857

    Citation

    De Franceschi, Lucia, et al. "Thrombosis and Sickle Cell Disease." Seminars in Thrombosis and Hemostasis, vol. 37, no. 3, 2011, pp. 226-36.
    De Franceschi L, Cappellini MD, Olivieri O. Thrombosis and sickle cell disease. Semin Thromb Hemost. 2011;37(3):226-36.
    De Franceschi, L., Cappellini, M. D., & Olivieri, O. (2011). Thrombosis and sickle cell disease. Seminars in Thrombosis and Hemostasis, 37(3), pp. 226-36. doi:10.1055/s-0031-1273087.
    De Franceschi L, Cappellini MD, Olivieri O. Thrombosis and Sickle Cell Disease. Semin Thromb Hemost. 2011;37(3):226-36. PubMed PMID: 21455857.
    * Article titles in AMA citation format should be in sentence-case
    TY - JOUR T1 - Thrombosis and sickle cell disease. AU - De Franceschi,Lucia, AU - Cappellini,Maria Domenica, AU - Olivieri,Oliviero, Y1 - 2011/03/31/ PY - 2011/4/2/entrez PY - 2011/4/2/pubmed PY - 2011/8/2/medline SP - 226 EP - 36 JF - Seminars in thrombosis and hemostasis JO - Semin. Thromb. Hemost. VL - 37 IS - 3 N2 - 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. SN - 1098-9064 UR - https://www.unboundmedicine.com/medline/citation/21455857/Thrombosis_and_sickle_cell_disease_ L2 - http://www.thieme-connect.com/DOI/DOI?10.1055/s-0031-1273087 DB - PRIME DP - Unbound Medicine ER -