System Maintenance Notice

Please note that maintenance work is scheduled between 4 AM and 5 AM - CET. During this time, you may experience reduced performance. Thanks for your patience.

Publications

Journal Article Review Epublish 36 citations

PubMed: 32722421 DOI: 10.3390/ijms21155279

Sickle Cell Disease: A Paradigm for Venous Thrombosis Pathophysiology.

Int J Mol Sci 21(15)

Authors: Maria A Lizarralde-Iragorri, Arun S Shet

Affiliations

  • Laboratory of Sickle Thrombosis and Vascular Biology, Sickle Cell Branch, National Institutes of Health, Bethesda, MD 20892, USA.

Abstract

Venous thromboembolism (VTE) is an important cause of vascular morbidity and mortality. Many risk factors have been identified for venous thrombosis that lead to alterations in blood flow, activate the vascular endothelium, and increase the propensity for blood coagulation. However, the precise molecular and cellular mechanisms that cause blood clots in the venous vasculature have not been fully elucidated. Patients with sickle cell disease (SCD) demonstrate all the risk factors for venous stasis, activated endothelium, and blood hypercoagulability, making them particularly vulnerable to VTE. In this review, we will discuss how mouse models have elucidated the complex vascular pathobiology of SCD. We review the dysregulated pathways of inflammation and coagulation in SCD and how the resultant hypercoagulable state can potentiate thrombosis through down-regulation of vascular anticoagulants. Studies of VTE pathogenesis using SCD mouse models may provide insight into the intersection between the cellular and molecular processes involving inflammation and coagulation and help to identify novel mechanistic pathways.

MeSH

Thrombophilia, Endothelium, Anemia, Sickle Cell, Venous Thrombosis, Risk Factors, Cells, Blood, Down-Regulation

Cited by