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beta-Antithrombin, subtype of antithrombin deficiency and the risk of venous thromboembolism in hereditary antithrombin deficiency: A family cohort study

Croles, F. N., Mulder, R., Mulder, A. B., Lukens, M. V. & Meijer, K., Aug-2018, In : Thrombosis Research. 168, p. 47-52 6 p.

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  • β-Antithrombin, subtype of antithrombin deficiency and the risk of venous thromboembolism in hereditary antithrombin deficiency

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DOI

Hereditary antithrombin deficiency is associated with a high incidence of venous thromboembolism (VTE), but VTE risk differs between families. Beta-antithrombin is reported to be the most active isoform of antithrombin in vivo. Whether beta-antithrombin activity and subtypes in antithrombin deficiency have impact on VTE risk has not been investigated outside the proband setting.

We performed a retrospective family cohort study to investigate whether subtypes of antithrombin deficiency or beta-antithrombin levels are associated with the risk of first or recurrent VTE.

Eighty-one subjects from 21 families were included, of which 52 were antithrombin deficient. Beta-antithrombin levels were decreased in most type I and type IIPE subjects, but normal levels were found in all subtypes of antithrombin deficiency.

The annual incidence of VTE in antithrombin-deficient family members was 1.24%, 95%CI: 0.72-1.99%, in low beta-antithrombin 1.36% (95%CI: 0.76-2.25%) and in normal beta-antithrombin 0.79% (95%CI: 0.10-2.77). The annual incidence of recurrence in family members was 3.1% (95%CI: 0.9-7.1%). Duration of anticoagulation had an impact on recurrence risk: In family members annual recurrence with fixed duration was 10% (95%CI: 2.1-29.2%), with indefinite duration 1.5% (95%CI: 0.2-5.4%), p <0.05. Beta-antithrombin levels were not associated with the risk for first or recurrent VTE in antithrombin deficient subjects.

Conclusions: In this high-risk antithrombin-deficient population, both subjects with low and normal plasma beta-antithrombin activity had high risks of first and recurrent VTE. This puts the importance of beta-antithrombin into question. Long-term anticoagulation is warranted in antithrombin-deficient VTE patients.

Original languageEnglish
Pages (from-to)47-52
Number of pages6
JournalThrombosis Research
Volume168
Publication statusPublished - Aug-2018

    Keywords

  • Natural anticoagulants, Antithrombin deficiency, beta-Antithrombin, Venous thrombosis, Pregnancy, DEEP-VEIN THROMBOSIS, PROTEIN-C, INHERITED THROMBOPHILIA, HEPARIN AFFINITY, SEQUENCE, GLYCOSYLATION, PREVALENCE, INHIBITORS, MUTATIONS, MORTALITY

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