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Review
. 2020 Jul 25;21(15):5279.
doi: 10.3390/ijms21155279.

Sickle Cell Disease: A Paradigm for Venous Thrombosis Pathophysiology

Maria A Lizarralde-Iragorri  1 , Arun S Shet  1
Affiliations
Review

Sickle Cell Disease: A Paradigm for Venous Thrombosis Pathophysiology

Maria A Lizarralde-Iragorri et al. Int J Mol Sci. .

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.

Keywords: endothelial injury; hypercoagulability; inflammation; mice models; sickle cell disease (SCD); stasis; venous thromboembolism (VTE).

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Conflict of interest statement

The authors declare no conflict of interest.

Figures

Figure 1
Figure 1
Sickle cell disease factors that contribute to thrombosis.
See this image and copyright information in PMC

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