Recombinant activated factor VII and tranexamic acid are haemostatically effective during major surgery in factor XI-deficient patients with inhibitor antibodies

Thrombosis and Haemostasis

Volumn 102, Issue 3, 2009, Pages 487-492

  Livnat, Tami ^a   Tamarin, Ilia ^a   Mor, Yoram ^a   Winckler, Harry ^a   Horowitz, Zeev ^a   Korianski, Yoseph ^a   Bar Zakay, Barak ^a   Seligsohn, Uri ^a   Salomon, Ophira ^a  

^a TEL AVIV UNIVERSITY   (Israel)

Author keywords

Factor XI; Factor XI inhibitor; Immunoglobulin A deficiency; Recombinant factor VIIa; Thrombin generation

Indexed keywords

BLOOD CLOTTING FACTOR; RECOMBINANT BLOOD CLOTTING FACTOR 7; THROMBIN; TRANEXAMIC ACID; UNCLASSIFIED DRUG;

ADULT; ARTICLE; BLEEDING; BLOOD CLOTTING FACTOR 11 DEFICIENCY; BLOOD SAMPLING; CASE REPORT; CHOLECYSTECTOMY; CONCENTRATION RESPONSE; CONTROLLED STUDY; DISEASE SEVERITY; DRUG EFFECT; FEMALE; HEMOSTASIS; HUMAN; IMMUNOGLOBULIN A DEFICIENCY; IN VITRO STUDY; INTRAOPERATIVE PERIOD; LASER LITHOTRIPSY; LOW DRUG DOSE; MAJOR SURGERY; MALE; PAROTIDECTOMY; POSTOPERATIVE COMPLICATION; PREOPERATIVE CARE; PRIORITY JOURNAL; PROSTATECTOMY; SINGLE DRUG DOSE; THROMBOSIS; TREATMENT OUTCOME; URETEROSCOPY;

ALLELES; BLOOD LOSS, SURGICAL; FACTOR VIIA; FACTOR XI; FACTOR XI DEFICIENCY; FEMALE; HEMOPHILIA A; HEMOSTASIS; HOMOZYGOTE; HUMANS; MALE; MIDDLE AGED; THROMBIN; THROMBOSIS; TIME FACTORS; TRANEXAMIC ACID;

EID: 70449381348     PISSN: 03406245     EISSN: None     Source Type: Journal    
DOI: 10.1160/TH09-03-0172     Document Type: Article

References (30)

1. Seligsohn U. High frequency of factor XI (PTA) deficiency in Ashkenazi Jews. Blood 1978; 51: 1223-1228. (Pubitemid 8344664)
2. Peretz H, Mulai A, Usher S, et al. The two common mutations causing factor XI deficiency in Jews stem from distinct founders: one of ancient Middle Eastern origin and another of more recent European origin. Blood 1997; 90: 2654-2659. (Pubitemid 27413475)
3. Salomon O, Zivelin A, Livnat T, et al. Prevalence, causes, and characterization of factor XI inhibitors in patients with inherited factor XI deficiency. Blood 2003; 101: 4783-4788. (Pubitemid 36857735)
4. Schulman S, Nemeth G. An illustrative case and a review on the dosing of recombinant factor VIIa in congenital factor XI deficiency. Hemophilia 2006; 12: 223-227.
5. Stern DM, Nossel HL, Owen J. Acquired antibody to factor XI in a patient with congenital FXI deficiency. J Clin Invest 1982; 69:1270-1276. (Pubitemid 12081054)
6. O'Connell NM, Riddell AF, Pascoe G, et al. Recombinant factor VIIa to prevent surgical bleeding in factor XI deficiency. Haemophilia 2008; 14: 775-781. (Pubitemid 352016034)
7. Avci Z, Malbora B, Gokdemir M, et al. Successful use of recombinant factor VIIa (NovoSeven) during cardiac surgery in a pediatric patient with congenital factor XI deficiency. Pediatr Cardiol 2008; 29: 220-222.
8. Livnat T, Zivelin A, Martinowitz U, et al. Prerequisites for recombinant factor VIIa-induced thrombin generation in plasmas deficient in factors VIII, IX, or XI. J Thromb Hemost 2006; 4:192-200.
9. Salomon O, Steinberg DM, Seligshon U. Variable bleeding manifestations characterize different types of surgery in patients with severe factor XI deficiency enabling parsimonious use of replacement therapy. Haemophilia 2006; 12: 490-493. (Pubitemid 44227591)
10. Mosnier LO, von dem Borne PA, Meijers JC, et al. Plasma TAFI levels influence the clot lysis time in healthy individuals in the presence of an intact intrinsic pathway of coagulation. Thromb Haemost 1998; 80: 829-835.
11. Hedner U. Factor VIIa in the treatment of haemophilia. Blood Coag Fibrinolysis 1990; 1: 307-317.
12. Billon S, Le Niger C, Escoffre-Barbe M, et al. 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. (Pubitemid 33022519)
13. Lawler P, White B. Pye S, et al. Successful use of recombinant factor VIIa in a patient with inhibitor secondary to severe factor XI deficiency. Hemophilia 2002; 8: 145-148. (Pubitemid 35238069)
14. Bern MM, Sahud M, Zhukov O, et al. Treatment of factor XI inhibitor using recombinant activated factor VIIa. Haemophilia 2005; 11: 20-25. (Pubitemid 40253763)
15. Brown SA. What dose of recombinant activated factor VII should be used in patients with factor XI deficiency? Haemophilia 2005; 11: 430-431.
16. Sucker C, Sabel M, Stummer W, et al. Recombinant factor VIIa for the prophylaxis of perioperative hemorrhage in a patient with congenital factor XI deficiency undergoing brain tumor neurosurgery. Clin Appl Thromb Hemost 2008; 14: 472-475.
17. Dempfle CE, Frietsch T, Hoffmann T, et al. Use of recombinant factor VIIa for hip replacement surgery in a patient with severe factor XI deficiency and drug-induced platelet defect. Haemophilia 2008; 14: 1147-1149.
18. Abshire T, Kenet G. Recombinant factor VIIa: review of efficacy, dosing regimens and safety in patients with congenital and acquired factor VIII or IX inhibitors. J Thromb Haemost 2004; 2: 899-909. (Pubitemid 40185737)
19. Abshire T, Kenet G. Safety update on the use of recombinant factor VIIa and the treatment of congenital and acquired deficiency of factor VIII or IX with inhibitors. Haemophilia 2008; 14: 898-902.
20. Monroe DM. Further understanding of recombinant activated factor VII mode of action. Semin Hematol 2008; 45: S7-S11. (Pubitemid 351787685)
21. Monroe DM, Hoffman M, Oliver JA, et al. Platelet activity of high dose factor VIIa is independent of tissue factor. Br J Haematol 1997; 99: 542-547. (Pubitemid 27519853)
22. Adams M. Assessment of thrombin generation: useful or hype? Semin Thromb Hemost 2009; 35: 104-110.
23. Van Veen JJ, Gatt A, Makris M. Thrombin generation testing in routine clinical practice: are we there yet? Br J Haematol 2008; 142: 889-903.
24. Berntorp E, Salvagno GL. Standardization and clinical utility of thrombin-generation assays. Semin Thromb Hemost 2008; 34: 670-682.
25. Hemker HC, Al Dieri R, De Smedt E, et al. Thrombin generation, a function test of the haemostatic-thrombotic system. Thromb Haemost 2006 ; 96: 553-561. (Pubitemid 44782533)
26. He S, Blombäck M, Jacobsson Ekman G, et al. The role of recombinant factor VIIa (FVIIa) in fibrin structure in the absence of FVIII/FIX. J Thromb Haemost 2003; 1: 1215-1219.
27. Lisman T, Mosnier LO, Lambert T, et al. Inhibition of fibrinolysis by recombinant factor VIIa in plasma from patients with severe hemophilia A. Blood 2002; 99: 175-179.
28. Wolberg AS, Allen GA, Monroe DM, et al. High dose factor VIIa improves clot structure and stability in a model of haemophilia B. Br J Haematol 2005; 131: 645-655.
29. Berliner S, Horowitz I, Martinowitz U, et al. Dental surgery in patients with severe factor XI deficiency without plasma replacement. Blood Coagul Fibrinolysis 1992; 4: 465-468.
30. Quelin F, Trossaërt M, Sigaud M, et al. 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. (Pubitemid 40185622)