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Prepublished online as a Blood First Edition Paper on February 13, 2003; DOI 10.1182/blood-2002-09-2794.
Submitted September 12, 2002
Accepted February 1, 2003
Prevalence, causes and characterization of factor XI inhibitors in patients with inherited factor XI deficiency
Ophira Salomon, Ariella Zivelin, Tami Levnat, Rima Dardik, Ron Loewenthal, Ophelia Avishai, David M Steinberg, Michael H Rosove, Niamh O'Connell, Christine A Lee, and Uri Seligsohn*
Thrombosis and Hemostasis Research Institute, Sheba Medical Center, Tel Hashomer, Israel
Tissue Typing Laboratory, Sheba Medical Center, Tel Hashomer, Israel
Department of Statistics, Tel Aviv University, Tel Aviv, Israel
Medicine, University of California, Los Angeles, CA, USA
Haemophilia Centre, Royal Free and University College Medical School, London, United Kingdom
* Corresponding author; email: seligson{at}sheba.health.gov.il.
Factor XI deficiency, an injury related bleeding disorder, is rare worldwide but common in Jews in whom two mutations, Glu117stop (type II) and Phe283Leu (type III) prevail. Mean factor XI activities in homozygotes for Glu117stop and homozygotes for Phe283Leu are 1 and 10 U/dL, respectively. Inhibitors to factor XI in patients with severe factor XI deficiency have been reported in a small number of instances. The objectives of this study were to determine the prevalence of acquired inhibitors against factor XI in patients with severe factor XI deficiency, discern whether they are related to a specific mutation(s) and characterize their activity. Clinical information was obtained from unrelated patients with severe factor XI deficiency and blood was analyzed for factor XI activity, inhibitor to factor XI and causative mutations. Immunoglobulin G purified from patients with an inhibitory activity was tested for binding to factor XI, effects on activation of factor XI by factor XIIa and thrombin, and activation of factor IX by exogenous factor XIa. Of 118 Israeli patients, 7 had an inhibitor all belonging to a subgroup of 21 homozygotes for Glu117stop who had a history of plasma replacement therapy. Three additional patients with inhibitors from U.K and U.S.A also had this genotype and were exposed to plasma. The inhibitors affected factor XI activation by thrombin or factor XIIa and activation of factor IX by factor XIa. The results imply that patients with a very low factor XI level are susceptible to development of an inhibitor following plasma replacement.
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