FXIII: Mechanisms of action in the treatment of hemophilia A

Journal of Thrombosis and Haemostasis

Volumn 12, Issue 2, 2014, Pages 159-168

  Rea, C J ^a   Foley, J H ^b   Okaisabor, O ^a   Sorensen B ^c  

^a KING'S COLLEGE LONDON   (United Kingdom)

^b UNIVERSITY OF BRITISH COLUMBIA   (Canada)

^c AARHUS UNIVERSITY HOSPITAL   (Denmark)

Author keywords

Blood coagulation; FXIII (28 41); Hemophilia A; Hemostasis; Therapeutics

Indexed keywords

BLOOD CLOTTING FACTOR 13; BLOOD CLOTTING FACTOR 8; BLOOD CLOTTING FACTOR 9; RECOMBINANT BLOOD CLOTTING FACTOR 9; THROMBIN;

ADULT; ANIMAL EXPERIMENT; ANIMAL MODEL; ARTICLE; BLOOD CLOTTING; BLOOD SAMPLING; CLINICAL ARTICLE; CONTROLLED STUDY; DRUG EFFICACY; DRUG SAFETY; ELECTRON MICROSCOPY; FIBRIN CLOT; FIBRINOLYSIS; HEMOPHILIA A; HEMOPHILIA B; HUMAN; MALE; MIDDLE AGED; MOUSE; NONHUMAN; PERMEABILITY; PRIORITY JOURNAL; THROMBOCYTE COUNT; UPREGULATION; YOUNG ADULT;

BLOOD COAGULATION; FXIII (28-41); HEMOPHILIA A; HEMOSTASIS; THERAPEUTICS;

ANIMALS; BLOOD COAGULATION; FACTOR XIII; HEMOPHILIA A; HUMANS; MALE; MICE; MICROSCOPY, ELECTRON;

EID: 84893573623     PISSN: 15387933     EISSN: 15387836     Source Type: Journal    
DOI: 10.1111/jth.12478     Document Type: Article

References (31)

1. Mann KG, Butenas S, Brummel K. The dynamics of thrombin formation. Arterioscler Thromb Vasc Biol 2003; 23: 17-25.
2. Brummel-Ziedins KE, Branda RF, Butenas S, Mann KG. Discordant fibrin formation in hemophilia. J Thromb Haemost 2009; 7: 825-32.
3. Foley JH, Nesheim ME, Rivard GE, Brummel-Ziedins KE. Thrombin activatable fibrinolysis inhibitor activation and bleeding in haemophilia A. Haemophilia 2012; 18: e316-22.
4. Wolberg AS. Thrombin generation and fibrin clot structure. Blood Rev 2007; 21: 131-42.
5. Bolton-Maggs PH, Pasi KJ. Haemophilias A and B. Lancet 2003; 361: 1801-9.
6. Manco-Johnson M. Comparing prophylaxis with episodic treatment in haemophilia A: implications for clinical practice. Haemophilia 2007; 13(Suppl. 2): 4-9.
7. UK Haemophilia Centre Doctor's Organisation (UKHCDO). Bleeding disorder statistics April 2011 to March 2012. UKHCDO. 2012
8. Valentino LA, Mamonov V, Hellmann A, Quon DV, Chybicka A, Schroth P, Patrone L, Wong WY. A randomized comparison of two prophylaxis regimens and a paired comparison of on-demand and prophylaxis treatments in hemophilia A management. J Thromb Haemost 2012; 10: 359-67.
9. Bjorkman S, Berntorp E. Pharmacokinetics of coagulation factors: clinical relevance for patients with haemophilia. Clin Pharmacokinet 2001; 40: 815-32.
10. Anthony LM, Rea CJ, Sorensen B. Inter and Intra-Individual Variation of Factor VIII Half-Life In Severe Haemophilia A. Blood (ASH Annual Meeting Abstracts) 2010; 116: Absract 544.
11. van den Berg HM, De Groot PH, Fischer K. Phenotypic heterogeneity in severe hemophilia. J Thromb Haemost 2007; 5(Suppl. 1): 151-6.
12. Foley JH, Petersen KU, Rea CJ, Harpell L, Powell S, Lillicrap D, Nesheim ME, Sorensen B. Solulin increases clot stability in whole blood from individuals and dogs with hemophilia. Blood 2012; 119: 3622-8.
13. Hvas AM, Sorensen HT, Norengaard L, Christiansen K, Ingerslev J, Sorensen B. Tranexamic acid combined with recombinant factor VIII increases clot resistance to accelerated fibrinolysis in severe hemophilia A. J Thromb Haemost 2007; 5: 2408-14.
14. Rea CJ, Foley JH, Ingerslev J, Sorensen B. Factor XIII combined with recombinant factor VIIa: a new means of treating severe haemophilia A. J Thromb Haemost 2011; 9: 510-6.
15. Rea CJ, Foley JH, Sorensen B. Factor XIII in the treatment of hemophilia A. N Engl J Med 2012; 366: 281-3.
16. Sorensen B, Johansen P, Christiansen K, Woelke M, Ingerslev J. Whole blood coagulation thrombelastographic profiles employing minimal tissue factor activation. J Thromb Haemost 2003; 1: 551-8.
17. Rea C, Foley J, Sorensen B. FXIII as a treatment in haemophilia. J Thromb Haemost 2011; 9: 510-6.
18. Ariens RA, Philippou H, Nagaswami C, Weisel JW, Lane DA, Grant PJ. The factor XIII V34L polymorphism accelerates thrombin activation of factor XIII and affects cross-linked fibrin structure. Blood 2000; 96: 988-95.
19. Hemker HC, Giesen P, Al Dieri R, Regnault V, de Smedt E, Wagenvoord R, Lecompte T, Beguin S. Calibrated automated thrombin generation measurement in clotting plasma. Pathophysiol Haemost Thromb 2003;33:4-15
20. Pastoft AE, Lykkesfeldt J, Ezban M, Tranholm M, Whinna HC, Lauritzen B. A sensitive venous bleeding model in haemophilia A mice: effects of two recombinant FVIII products (N8 and Advate((R))). Haemophilia 2012; 18: 782-8.
21. Bolton-Maggs PH. The rare inherited coagulation disorders. Pediatr Blood Cancer 2013; 60: S36-40.
22. Fraser SR, Booth NA, Mutch NJ. The antifibrinolytic function of factor XIII is exclusively expressed through alpha(2)-antiplasmin cross-linking. Blood 2011; 117: 6371-4.
23. Sakata Y, Aoki N. Significance of cross-linking of alpha 2-plasmin inhibitor to fibrin in inhibition of fibrinolysis and in hemostasis. J Clin Invest 1982; 69: 536-42.
24. Wolberg AS, Campbell RA. Thrombin generation, fibrin clot formation and hemostasis. Transfus Apher Sci 2008; 38: 15-23.
25. Larsen OH, Poulsen L, Sorensen B, Rea CJ. FXIII levels in Haemophilia. Haemophilia 2012; 18(Suppl. 2): 36.
26. Cushman M, O'Meara ES, Folsom AR, Heckbert SR. Coagulation factors IX through XIII and the risk of future venous thrombosis: the Longitudinal Investigation of Thromboembolism Etiology. Blood 2009; 114: 2878-83.
27. Bereczky Z, Muszbek L. Factor XIII and venous thromboembolism. Semin Thromb Hemost 2011; 37: 305-14.
28. Kalaria VG, Zareba W, Moss AJ, Pancio G, Marder VJ, Morrissey JH, Weiss HJ, Sparks CE, Greenberg H, Dwyer E, Goldstein R, Watelet LF. Gender-related differences in thrombogenic factors predicting recurrent cardiac events in patients after acute myocardial infarction The THROMBO Investigators. Am J Cardiol 2000; 85: 1401-8.
29. Astermark J, Donfield SM, DiMichele DM, Gringeri A, Gilbert SA, Waters J, Berntorp E. A randomized comparison of bypassing agents in hemophilia complicated by an inhibitor: the FEIBA NovoSeven Comparative (FENOC) Study. Blood 2007; 109: 546-51.
30. Berntorp E. Differential response to bypassing agents complicates treatment in patients with haemophilia and inhibitors. Haemophilia 2009; 15: 3-10.
31. Visich JE, Zuckerman LA, Butine MD, Gunewardena KA, Wild R, Morton KM, Reynolds TC. Safety and pharmacokinetics of recombinant factor XIII in healthy volunteers: a randomized, placebo-controlled, double-blind, multi-dose study. Thromb Haemost 2005; 94: 802-7.