Thrombin generation and platelet activation induced by rFVIIa (NovoSeven®) and NN1731 in a reconstituted cell-based model mimicking haemophilia conditions

Haemophilia

Volumn 15, Issue 6, 2009, Pages 1318-1326

  Aljamali, M N ^a   Kjalke, M ^a   Hedner, U ^a   Ezban, M ^a   Tranholm, M ^a  

^a NOVO NORDISK A S   (Denmark)

Author keywords

Moderate and mild haemophilia A or B; NN1731; Platelet activation; RFVIIa; Severe haemophilia A or B; Thrombin generation

Indexed keywords

BLOOD CLOTTING FACTOR; BLOOD CLOTTING FACTOR 8; BLOOD CLOTTING FACTOR 9; NN 1731; RECOMBINANT BLOOD CLOTTING FACTOR 7A; THROMBIN; UNCLASSIFIED DRUG;

ARTICLE; CONCENTRATION RESPONSE; DISEASE MODEL; DRUG MECHANISM; FLOW CYTOMETRY; HEMOPHILIA; HEMOSTASIS; HUMAN; HUMAN CELL; PRIORITY JOURNAL; THROMBOCYTE ACTIVATION;

BLOOD COAGULATION FACTORS; CELLS, CULTURED; DOSE-RESPONSE RELATIONSHIP, DRUG; FACTOR VII; FACTOR VIIA; HEMOPHILIA A; HEMOPHILIA B; HUMANS; PLATELET ACTIVATION; RECOMBINANT PROTEINS; THROMBIN;

EID: 70449555316     PISSN: 13518216     EISSN: 13652516     Source Type: Journal    
DOI: 10.1111/j.1365-2516.2009.02073.x     Document Type: Article

References (28)

1. Schwaab R, Brackmann HH, Meyer C. Haemophilia A: mutation type determines risk of inhibitor formation. Thromb Haemost 1995, 74:1402-6.
2. Jacquemin MG, De Maeyer M, D'Oiron R. Molecular mechanism of mild and moderate hemophilia A. J Thromb Haemost 2003, 1:456-63.
3. Hedner U. Treatment of patients with factor VIII and factor IX inhibitors with special focus on the use of recombinant factor VIIa. Thromb Haemost 1999, 86:531-9.
4. Wolberg AS, Allen GA, Monroe DM, Hedner U, Roberts HR, Hoffman M. High dose factor VIIa improves clot structure and stability in a model of hemophilia B. Br J Haematol 2005, 131:645-55.
5. D'Oiron R, Volot F, Reynaud J. Impact of choice of treatment for bleeding episodes on inhibitor outcome in patients with mild/moderate hemophilia A and inhibitors. Semin Haematol 2006, 43(Suppl 1):S3-9.
6. D'Oiron R. Prevention and treatment of inhibitor development in mild/moderate hemophilia A (MHA) patients. Haemophilia 2008, 14:1-120.
7. Leebeek FWG, Kappers-Klunne MC, Jie KSG. Effective and safe use of recombinant factor VIIa (NovoSeven) in elderly mild haemophilia A patients with high-titer antibodies against factor VIII. Haemophilia 2004, 10:250-3.
8. Haya S, Moret A, Cid AR. Inhibitors in haemophilia A: current management and open issues. Haemophilia 2007, 13(Suppl. 5):52-60.
9. Hoffman M, Monroe DM. A cell-based model of haemostasis. Thromb Haemost 2001, 85:958-65.
10. Hoffman M. A cell-based model of coagulation and the role of factor VIIa. Blood Rev 2003, 17:S1-5.
11. Kjalke M, Monroe DM, Hoffman M, Roberts HR, Hedner U, Ezban M. High-dose factor VIIa restores platelet activation and total thrombin generation in a model system mimicking hemophilia A or B conditions. Thromb Haemost 1999, S303.
12. Tranholm M, Kristensen K, Kristensen AT. Improved haemostasis with superactive analogs of factor VIIa in a mouse model of hemophilia A. Blood 2003, 102:3615-20.
13. Allen GA, Persson E, Campbell RA, Ezban M, Hedner U, Wolberg AS. A variant of recombinant factor VIIa with enhanced procoagulant and antifibrinolytic activities in an in vitro model of hemophilia. Arterioscler Thromb Vasc Biol 2007, 27:683-9.
14. Lindley CM, Sawyer WT, Macik BG. Pharmacokinetics and pharmacodynamics of recombinant factor VIIa. Clin Pharmacol Therapeutics 1994, 55:638-48.
15. Kjalke M, Ezban M, Monroe DM, Hoffman M, Roberts HR, Hedner U. High-dose factor VIIa increases initial thrombin generation and mediates faster platelet activation in thrombocytopenia-like conditions in a cell-based model system. Br J Haematol 2001, 114:114-20.
16. Allen GA, Wolberg AS, Oliver JA, Hoffman M, Roberts HR, Monroe DM. Impact of procoagulant concentration on rate, peak and total thrombin generation in a model system. J Thromb Haemost 2004, 2:402-13.
17. Van Den Berg HM, De Groot PHG, Fischer K. Phenotypic heterogeneity in severe hemophilia. J Thrombos Haemost 2007, 5:151-6.
18. Young G, Shafer FE, Rojas P, Seremetis S. Singe 270 μg kg-1-dose rFVIIa vs. standard 90 μg kg-1-dose rFVIIa and APCC for home treatment of joint bleeds in haemophilia patients with inhibitors: a randomized comparison. Haemophilia 2008, 14:287-94.
19. Kenet G, Martinowitz U. Single-dose recombinant activated factor VII therapy in hemophilia patients with inhibitors. Semin Hematol 2008, 45(Suppl 1):S38-41.
20. Gabriel DA, Li X, Monroe DM, Roberts HR. Recombinant human factor VIIa (rFVIIa) can activate factor FIX on activated platelets. J Thromb Haemost 2004, 2:1816-22.
21. Ljung R, Petrini P, Tengborn L. Haemophilia B mutations in Sweden: a population-based study of mutational heterogeneity. Haemophilia 2001, 113:81-6.
22. Jenkins PV, Egan H, Keenan C. Mutation analysis of haemophilia B in the Irish population: increased prevalence caused by founder effect. Haemophilia 2008, 14:717-22.
23. Lusher JM. Inhibitor antibodies to factor VIII and factor IX: management. Semin Thromb Haemost 2000, 26:179-88.
24. Blomback B, Carlsson K, Fatah K, Hessel B, Procyk R. Fibrin in human plasma: gel architectures governed by rate and nature of fibrinogen activation. Thromb Res 1994, 75:521-38.
25. Weisel JW, Veklich Y, Collet JP, Francis CW. Structural studies of fibrinolysis by electron and light microscopy. Thromb Haemost 1999, 82:277-82.
26. Wolberg AS, Monroe DM, Roberts HR, Hoffman M. Elevated prothrombin results in clots with an altered fibrin structure: a possible mechanism of the increased thrombotic risk. Blood 2003, 101:3008-13.
27. Gabriel DA, Muga K, Boothroyd EM. The effect of fibrin structure on fibrinolysis. J Biol Chem 1992, 267:24259-63.
28. Collet JP, Park D, Lesty C. Influence of fibrin network conformation and fibrin fiber diameter on fibrinolysis speed. Thromb Vasc Biol 2000, 20:1354-61.