A mutation in factor XIII (Val34Leu) was reported to protect against Venous
thromboembolism. We evaluated the effect of Val34Leu on thrombotic risk in
352 factor V Leiden carriers who were first-degree relatives of 132 thromb
otic propositi carrying factor V Leiden. The total observation period was 2
594 years in 92 Val34Leu carriers and 7444 years in 260 non-carriers. The a
nnual incidence of a first episode of venous thromboembolism was 0.31% in V
al34Leu carriers and 0.44% in non-carriers [relative risk (RR) for Venous t
hromboembolism: 0.7, 95% CI 0.3-1.5]. Age-specific RR for Venous thromboemb
olism were (for Val34Leu carriers and non-carriers respectively): 1.0 (95%
CT 0.3-3.2) in the age group of 15-30 years, 0.4 (95% CI 0.05-3.0) in the a
ge group of 30-45 years, 0.6 (95% CI 0.1-2.9) in the group aged 45-60 years
and 0.5 (95% CI 0.06-4.5) in relatives order than 60 years. In conclusion,
the impact of FXIII Val34Leu on the venous thromboembolic risk is modest,
suggesting that screening for this mutation in factor V Leiden carriers is
not justified.