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

Steven P. Grover

doi:10.1055/s-0044-1791779

Seminars in Thrombosis and Hemostasis, Table of Contents

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

Authors

Steven P. Grover

¹UNC Blood Research Center, The University of North Carolina at Chapel Hill, Chapel Hill, North Carolina

²Division of Hematology, Department of Medicine, The University of North Carolina at Chapel Hill, Chapel Hill, North Carolina

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

Full Text

References

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