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Semin Thromb Hemost 2025; 51(03): 322-328
DOI: 10.1055/s-0044-1791779
Review Article

Hereditary Angioedema and Venous Thromboembolism: Where There's Smoke, There's Fire

Steven P. Grover
1   UNC Blood Research Center, The University of North Carolina at Chapel Hill, Chapel Hill, North Carolina
2   Division of Hematology, Department of Medicine, The University of North Carolina at Chapel Hill, Chapel Hill, North Carolina
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Funding This work was supported by a Scholar Award from the American Society of Hematology and R01HL171042 from the National Heart, Lung, and Blood Institute of the National Institutes of Health to S.P.G. The content is solely the responsibility of the authors and does not necessarily represent the official views of the National Institutes of Health.
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Abstract

C1-inhibitor deficiency–associated hereditary angioedema (C1INH-HAE) is a rare congenital swelling disorder caused by mutations in the SERPING1 gene. Despite evidence of a systemic procoagulant state in C1INH-HAE, dogma held that this disorder was not associated with thrombotic pathologies. Recent population scale epidemiological evidence has directly challenged this, with C1INH-HAE being associated with a significantly increased risk of venous thromboembolism (VTE). This review considers the growing body of evidence supporting associations between HAE and both a systemic procoagulant state and an increased risk of VTE. In the setting of C1INH-HAE, the relationship between the observed procoagulant and thrombotic phenotypes is a prime example of “where there's smoke, there's fire.” This review also discusses the impact of C1INH-HAE disease modifying therapies on coagulation and VTE. Further, the utility of preclinical mouse models of C1-inhibitor deficiency is considered.

Keywords

blood coagulation factors - C1-inhibitor protein - hereditary angioedema - venous thrombosis - venous thromboembolism


Publikationsverlauf

Artikel online veröffentlicht:
17. Oktober 2024

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