Seminars in Hematology
Volume 43 , Pages S10-S12 , January 2006

Inhibitors to Factor XI in Patients With Severe Factor XI Deficiency

  • Ophira Salomon

      Affiliations

    • Corresponding Author InformationAddress correspondence to Ophira Salomon, MD, Senior Hematologist, Amalia Biron Research Institute of Thrombosis and Hemostasis, Chaim Sheba Medical Center, Tel Hashomer and Sackler Faculty of Medicine, Tel Aviv University, 52621 Israel
    • ,
  • Ariella Zivelin
    • ,
  • Tami Livnat
    • ,
  • Uri Seligsohn

References 

  1. Baglia FA , Shrimpton CN , Emsley J , Kitagawa K , Ruggeri ZM , Lopez JA , et al.  Factor XI interacts with leucine-rich repeats of glycoprotein Ibα on the activated platelet . J Biol Chem . 2004;279:49323–49329
  2. von dem Borne PAK , Meijers JCM , Bouma BN . Feedback activation of factor XI by thrombin in plasma results in additional formation of thrombin that protects fibrin clots from fibrinolysis . Blood . 1995;86:3035–3042
  3. Asakai R , Chung DW , Davie EW , Seligsohn U . Factor XI deficiency in Ashkenazi Jews in Israel . N Engl J Med . 1991;325:153–158
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  5. Kravtsov DV , Wu W , Meijers JCM , Sun MF , Blinder MA , Dang TP , et al.  Dominant factor XI deficiency caused by mutations in the factor XI catalytic domain . Blood . 2004;104:128–134
  6. Salomon O , Zivelin A , Livnat T , Dardik R , Loewenthal R , Avishai O , et al.  Prevalence, causes, and characterization of factor XI inhibitors in patients with inherited factor XI deficiency . Blood . 2003;101:4783–4788
  7. Stern DM , Nossel HL , Owen J . Acquired antibody to factor XI in a patient with congenital factor XI deficiency . J Clin Invest . 1982;69:1270–1276
  8. De La Cadena RA , Baglia FA , Johnson CA , Wenk RE , Amernick R , Walsh PN , et al.  Naturally occurring human antibodies against two distinct functional domains in the heavy chain of FXI/FXIa . Blood . 1988;72:1748–1754
  9. Goldsmith GH , Silverman P . Inhibitors of plasma thromboplastin antecedent (factor XI) (Studies on mechanism of inhibition) . J Lab Clin Med . 1985;106:279–285
  10. Quelin F , Trossaert M , Sigaud M , Mazancourt PD , Fressinaud E . Molecular basis of severe factor XI deficiency in seven families from the West of France. Seven novel mutations, including an ancient Q88X mutation . J Thromb Haemost . 2004;2:71–76
  11. Zadra G , Asselta R , Malcovati M , Santagostino E , Peyvandi F , Mannucci PM , et al.  Molecular genetic analysis of severe coagulation factor XI deficiency in six Italian patients . Haematologica . 2004;89:1332–1340
  12. Sato E , Kawamata N , Kato A , Oshimi K . A novel mutation that leads to a congenital factor XI deficiency in a Japanese family . Am J Hematol . 2000;63:165–169
  13. Iijima K , Udagawa A , Kawasaki H , Murakami F , Shimomura T , Ikawa S . A factor XI deficiency associated with a nonsense mutation (Trp501stop) in the catalytic domain . Br J Haematol . 2000;111:556–558
  14. Ventura C , Santos AI , Tavares A , Gago T , Lavinha J , McVey JH , et al.  Molecular genetic analysis of factor XI deficiency (Identification of five novel gene alterations and the origin of type II mutation in Portuguese families) . Thromb Haemost . 2000;84:833–840
  15. Bolton-Maggs PH , Peretz H , Butler R , Mountford R , Keeney S , Zacharski L , et al.  A common ancestral mutation (C128X) occurring in 11 non-Jewish families from the UK with factor XI deficiency . J Thromb Haemost . 2004;2:918–924
  16. Hay CR , Ollier W , Pepper L , Cumming A , Keeney S , Goodeve AC , et al.  UKHCDO Inhibitor Working Party   HLA class II profile (A weak determinant of factor VIII inhibitor development in severe haemophilia A) . Thromb Haemost . 1997;77:234–237
  17. Teruya J , Styler M . Management of factor XI inhibitor for cardiac intervention (Successful treatment with immunosuppressive therapy and plasma exchange) . Haemophilia . 2000;6:158–161
  18. Connelly NR , Brull SJ . Anesthetic management of a patient with factor XI deficiency and factor XI inhibitor undergoing a cesarean section . Anesth Analg . 1993;76:1365–1366
  19. Hedner U . Factor VIIa in the treatment of haemophilia . Blood Coag Fibrinolysis . 1990;1:307–317
  20. Billon S , Le Niger C , Escoffre-Barbe M , Vicariot M , Abgrall JF . The use of recombinant factor VIIa (NovoSeven) in a patient with a factor XI deficiency and circulating anticoagulant . Blood Coagul Fibrinolysis . 2001;12:551–553
  21. Lawler P , White B , Pye S , Hermans C , Riddell A , Costello C , et al.  Successful use of recombinant factor VIIa in a patient with inhibitor secondary to severe factor XI deficiency . Hemophilia . 2002;8:145–148
  22. Bern MM , Sahud M , Zhukov O , Qu K , Mitchell W . Treatment of factor XI inhibitor using recombinant activated factor VIIa . Haemophilia . 2005;11:20–25
  23. Salomon O , Steinberg DM , Dardik R , Rosenberg N , Zivelin A , Tamarin I , et al.  Inherited factor XI deficiency confers no protection against acute myocardial infarction . J Thromb Haemost . 2003;1:658–661
  24. Livnat O , Salomon O , Zivelin A , Seligsohn U . The severely impaired thrombin generation in 5 patients with severe factor XI deficiency and inhibitors is corrected in vitro by recombinant VIIa. Thromb Haemost. 2003;89: (abstr 1188)

PII: S0037-1963(05)00225-8

doi: 10.1053/j.seminhematol.2005.11.018

Seminars in Hematology
Volume 43 , Pages S10-S12 , January 2006

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