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Cardiology 2007;107:69–72

Percutaneous Coronary Intervention in a Patient with Congenital Factor XI Deficiency and Acquired Inhibitor

Mungee S. · Lapin R. · Cavusoglu E. · Clark L.T. · Marmur J.D.

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Division of Cardiology, SUNY Downstate Medical Center, Brooklyn, N.Y., USA

Corresponding Author

Jonathan D. Marmur, MD

Director of Cardiac Catheterization and Interventional Cardiology

SUNY Health Science Center at Brooklyn, 450 Clarkson Avenue, Box 1257

Brooklyn, NY 11203-2098 (USA)

Tel. +1 718 270 3273, Fax +1 718 270 4503, E-Mail jonathan@marmur.com

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Background: Factor XI deficiency has been associated with bleeding diathesis mostly secondary to trauma and post-operatively depending on the severity of deficiency. Cases with factor XI deficiency having undergone cardiac surgery and coronary intervention after appropriate replacement therapy have been reported in the past. The presence of inhibitor in factor XI deficiency poses a hematological challenge and literature regarding coronary intervention in such patients is limited. Immunosuppressive therapy, plasma exchange and factor VII product transfusions have been used prior to cardiac interventions in few such reported cases. Method: We report our approach in such a case of Percutaneous Transluminal Coronary Angioplasty in a 72-year-old male of Jewish origin who has congenital factor XI deficiency complicated with acquired inhibitor. Results: In some cases, the acuity of the coronary syndrome may mandate immediate coronary intervention. However, patient’s history of factor XI deficiency and acquired inhibitor pose a major dilemma of further course of action. We performed percutaneous balloon angioplasty in this case with no anti-coagulant and with favorable outcome. Conclusion: Under these circumstances of significant coagulation disorder and based on the case report, we recommend that balloon angioplasty be undertaken with no additional anti-coagulation other than Aspirin.

© 2007 S. Karger AG, Basel


  1. 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.
  2. Seligsohn U: Factor XI deficiency. Thromb Haemost1993;70:68–71.
  3. Rosenthal RL, Dreskin OH, Rosenthal N: New hemophilia like disease caused by deficiency of a third plasma thromboplastin factor. Proc Soc Exp Biol Med 1953;82:171.
  4. Bolton-Maggs PHB, Young-Wan-Yin R, McCraw A, Slack J, Kernoff PBA: Inherience and bleeding in factor XI deficiency. Br J Haematol 1988;69:521–528.
  5. Asakai R, Chung DW, Davie EW, Seligsohn U: Factor XI in Ashkenazi Jews in Israel. New Engl J Med 1991;325:153–158.
  6. Biggs R, Sharp AA, Margolis J, Hardisty RM, Stewart J, Davidson WM: Defects in the early stages of blood coagulation: a report of four cases. Br J Haematol 1958;4:177–192.
  7. Ragni MV, Sinha D, Seaman F, Lewis JH, Spero JA, Walsh PW: Comparison of bleeding tendency, factor XI coagulant activity, and factor XI antigen in 25 factor XI-deficient kindreds. Blood 1985;65:719–724.
  8. Saito H, Ratnoff OD, Bouma BN, Seligsohn U: Failure to detect variant (CRM+) plasma thromboplastin antecedent (PTA, factor XI) molecules in hereditary deficiency: a study of 125 patients of several ethnic backgrounds. J Lab Clin Med 1985;106:718–722.
  9. Asakai R, Chung DW, Ratnoff OD, Davie EW: Factor XI (plasma thromboplastin antecedent) deficiency in Ashkenazi Jews is a bleeding disorder that can result from three types of point mutations. Proc Natl Acad Sci USA 1989;86:7667–7671.
  10. Bolton-Maggs PHB, Wensley RT, Kernoff PBA, Kasper CK, Winkelman L, Lane RS, Smith JK: Production and therapeutic use of a factor XI concentrate from plasma. Thromb Haemostas 1992;67:314–319.
  11. Gitel SN, Varon D, Schulman S, Martinowitz U: Clinical experiences of a FXI concentrate: possible side effects. Thromb Hemostas 1991;65:1157.
    External Resources
  12. Hedner U: Factor VIIa in the treatment of hemophilia. Blood Coagul Fibrinolysis 1990;1:307–317.
  13. Billion S, Le Niger C, Escoffire-Barbe M, Vicariot M, Abgrall JF: The use of recombinant factor VIIa (NovoSeven) in a patient with a factor XI deficiency and a circulating anticoagulant. Blood Coagul Fibrinolysis 2001;12:551–553.
  14. Musso R, Cultrera G, Giuiffridia G, Fichera E, Di Franscesco E, Salemi S, et al: Recombinant activated factor VII as haemostatic agent in homozygous factor XI deficiency patients. Thromb Haemost 2001;12:551–553.
  15. Brunken R, Folletee D, Wittig J: Coronary artery bypass in hereditary factor XI deficiency. Ann Thorac Surg 1984;38:406–408.
  16. Vander Woude J, Milam J, Walker W, Houchin P, Weiland AP, Cooley DA: Cardiovascular surgery in patients with congenital plasma coagulopathies. Ann Thorac Surg 1998;46:283–288.
    External Resources
  17. Nawabi IU, Garcia A, Mitter A, Brunner RE: Anticoagulation during CABG in factor XI deficiency a case report: Thromb Haemost 1994;71:261.
  18. 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.

Article / Publication Details

First-Page Preview
Abstract of Case Report

Received: March 27, 2006
Accepted: April 06, 2006
Published online: June 22, 2006

Number of Print Pages: 4
Number of Figures: 1
Number of Tables: 0

ISSN: 0008-6312 (Print)
eISSN: 1421-9751 (Online)

For additional information: https://www.karger.com/CRD