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Mortality and causes of death in families with the factor V Leiden mutation
(resistance to activated protein C)
ET Hille, RG Westendorp, JP Vandenbroucke and FR Rosendaal
Department of Clinical Epidemiology, University Hospital Leiden, The
Netherlands.
To investigate whether resistance to activated protein C (APC resistance)
because of a mutation in the factor V gene (factor V Leiden) leads to a
decrease in life expectancy, we analyzed overall and cause-specific
mortality in 171 parents whose offspring carried this mutation. Compared
with the Dutch general population, and after adjustment for age, sex, and
calendar period, we found no excess deaths in the parents (standardized
mortality ratio [SMR], 1.0; 95% confidence interval [CI], 0.8 to 1.2). The
cause-specific SMR for malignant neoplasms (1.0; 95% CI, 0.6 to 1.4),
diseases of the circulatory system (1.0; 95% CI, 0.7 to 1.4), and
cerebrovascular disease (1.0; 95% CI, 0.4 to 1.9) also did not differ from
unity. The SMRs for diseases of the respiratory system (1.4; 95% CI, 0.6 to
2.6) and for ischemic heart diseases (1.1; 95% CI, 0.7 to 1.7) were
slightly increased. Under the age of 45 years, there was a ninefold
increase of dying from ischemic heart disease. Thromboembolic complications
were mentioned only once (venous embolism or thrombosis) as an underlying
("primary") cause of death (SMR, 2.3; 95% CI, 0.1 to 13.0) and three times
(pulmonary embolisms) as a contributing ("secondary") cause of death (SMR,
1.5; 95% CI, 0.3 to 4.4). We conclude that there is no major effect of APC
resistance on life expectancy. Therefore, long-term anticoagulation in
carriers of factor V Leiden, on the basis of the carrier state alone, is
not indicated.
Volume 89,
Issue 6,
pp. 1963-1967,
03/15/1997
Copyright © 1997 by The American Society of Hematology
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