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Blood, 15 April 2008, Vol. 111, No. 8, pp. 4113-4117.
Prepublished online as a Blood First Edition Paper on February 11, 2008; DOI 10.1182/blood-2007-10-120139.
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Submitted October 25, 2007
Accepted February 6, 2008
Reduced incidence of ischemic stroke in patients with severe factor XI deficiency
Ophira Salomon, David M. Steinberg, Nira Koren-Morag, David Tanne, and Uri Seligsohn*
Deptartment of Hematology, The Amalia Biron Research Institute of Thrombosis and Hemostasis, Sheba Medical Center, Tel Hashomer and Sackler Faculty of Medicine, Tel Aviv University Israel, Tel Hashomer, Israel
Department of Statistics and Operations Research, Raymond and Beverly Sackler Faculty of Exact Sciences, Tel Aviv University, Tel Aviv, Israel
Division of Epidemiology and Preventive Medicine, Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel
Stroke Unit, Department of Neurology, Sheba Medical Center, Tel Hashomer and Sackler Faculty of Medicine, Tel Aviv University, Tel Hashomer, Israel
* Corresponding author; email: seligson{at}sheba.health.gov.il.
Inherited disorders of hemostasis are natural models for investigating mechanisms of thrombosis and development of antithrombotic therapy. Because mice with total factor XI deficiency are protected against ischemic stroke and do not manifest excessive bleeding, we investigated the incidence of ischemic stroke in patients with severe inherited factor XI deficiency. Incidence of ischemic stroke in 115 patients aged 45 years or more with severe factor XI deficiency (activity less than 15 U/dL) was compared to incidence in the Israeli population as estimated from a stroke survey of 1528 patients. Adjustment for major risk factors of stroke (hypertension, diabetes mellitus, hypercholesterolemia, current smoking) was based on comparison of their prevalence in the stroke survey to an Israeli health survey of 9509 subjects. Incidence of myocardial infarction in the factor XI cohort was also recorded. After adjustment for the four major risk factors of ischemic stroke, the expected incidence of ischemic stroke was 8.56 compared to one observed (p = 0.0037). The reduced 1:115 incidence of ischemic stroke contrasted with a 19:115 incidence of myocardial infarction, similar to the expected incidence. Thus, severe factor XI deficiency probably is protective against ischemic stroke but not against acute myocardial infarction.
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