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Prepublished online as a Blood First Edition Paper on February 13, 2003; DOI 10.1182/blood-2002-09-2794. This Article Full Text Full Text (PDF) All Versions of this Article: 2002-09-2794v1 101/12/4783    most recent Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Rights and Permissions Citing Articles Citing Articles via HighWire Citing Articles via CrossRef Citing Articles via Google Scholar Google Scholar Articles by Salomon, O. Articles by Seligsohn, U. Search for Related Content PubMed PubMed Citation Articles by Salomon, O. Articles by Seligsohn, U. Related Collections Hemostasis, Thrombosis, and Vascular Biology Immunobiology Clinical Trials and Observations Related Article in Blood Online Social Bookmarking                   What's this?  Previous Article  |  Table of Contents  |  Next Article  Blood, 15 June 2003, Vol. 101, No. 12, pp. 4783-4788 HEMOSTASIS, THROMBOSIS, AND VASCULAR BIOLOGY Prevalence, causes, and characterization of factor XI inhibitors in patients with inherited factor XI deficiency Ophira Salomon, Ariella Zivelin, Tami Livnat, Rima Dardik, Ron Loewenthal, Ophelia Avishai, David M. Steinberg, Michael H. Rosove, Niamh O'Connell, Christine A. Lee, and Uri Seligsohn From the Thrombosis and Hemostasis Research Institute, Tissue Typing Laboratory, Sheba Medical Center, Tel Hashomer, Israel; Sackler Faculty of Medicine, Tel Aviv University, Israel; Department of Statistics and Operations Research, Raymond and Beverley Sackler Faculty of Exact Sciences, Tel Aviv University, Israel; Division of Hematology-Oncology, Department of Medicine, University of California, Los Angeles; and Haemophilia Centre and Haemostasis Unit, Royal Free and University College Medical School, London, United Kingdom. Factor XI deficiency, an injury-related bleeding disorder, is rare worldwide but common in Jews in whom 2 mutations, Glu117Stop (type II) and Phe283Leu (type III), prevail. Mean factor XI activities in homozygotes for Glu117Stop and 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. This study was undertaken to determine the prevalence of acquired inhibitors against factor XI in patients with severe factor XI deficiency, discern whether these inhibitors are related to specific mutations, 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 belonged to a subgroup of 21 homozygotes for Glu117Stop who had a history of plasma replacement therapy. Three additional patients with inhibitors from the United Kingdom and the United States 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. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? Related Article in Blood Online: Factor XI inhibitors David Gailani Blood 2003 101: 4649. [Full Text] [PDF] This article has been cited by other articles: O. Salomon, I. Tamarin, T. Livnat, Z. Horovitz, J. Kuriansky, U. Martinowitz, Y. Mor, and U. Seligsohn Patients with Severe Factor XI Deficiency Who Have An Inhibitor or IgA Deficiency Can Undergo Uneventful Major Surgery by a Single Infusion of Low Dose Recombinant Factor VIIa and Use of Tranexamic Acid. Blood (ASH Annual Meeting Abstracts), November 16, 2008; 112(11): 1215 - 1215. [Abstract] G. Zadra, R. Asselta, M. L. Tenchini, G. Castaman, U. Seligsohn, P. M. Mannucci, and S. Duga Simultaneous genotyping of coagulation factor XI type II and type III mutations by multiplex real-time polymerase chain reaction to determine their prevalence in healthy and factor XI-deficient Italians Haematologica, May 1, 2008; 93(5): 715 - 721. [Abstract] [Full Text] [PDF] M. Ranucci, G. Isgro, G. Soro, D. Conti, and B. De Toffol Efficacy and Safety of Recombinant Activated Factor VII in Major Surgical Procedures: Systematic Review and Meta-analysis of Randomized Clinical Trials Arch Surg, March 1, 2008; 143(3): 296 - 304. [Abstract] [Full Text] [PDF] M. Zucker, A. Zivelin, J. Teitel, and U. Seligsohn Induction of an inhibitor antibody to factor XI in a patient with severe inherited factor XI deficiency by Rh immune globulin Blood, February 1, 2008; 111(3): 1306 - 1308. [Abstract] [Full Text] [PDF] W. K. Hoots Challenges in the Therapeutic Use of a "So-Called" Universal Hemostatic Agent: Recombinant Factor VIIa Hematology, January 1, 2006; 2006(1): 426 - 431. [Abstract] [Full Text] [PDF] H. R. Roberts, D. M. Monroe, and G. C. White The use of recombinant factor VIIa in the treatment of bleeding disorders Blood, December 15, 2004; 104(13): 3858 - 3864. [Abstract] [Full Text] [PDF] P. M. Mannucci, S. Duga, and F. Peyvandi Recessively inherited coagulation disorders Blood, September 1, 2004; 104(5): 1243 - 1252. [Abstract] [Full Text] [PDF] M. E. Rick, C. E. Walsh, and N. S. Key Congenital Bleeding Disorders Hematology, January 1, 2003; 2003(1): 559 - 574. [Abstract] [Full Text] [PDF]

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