Thrombosis Research
Volume 125 , Pages S63-S66 , April 2010

Hemophilic Synovitis: Factor VII and the Potential Role of Extravascular Factor VIIa

  • Paul E. Monahan

      Affiliations

    • Pediatric Hematology/Oncology, University of North Carolina at Chapel Hill, USA
    • Harold R. Roberts Hemophilia Diagnostic and Treatment Center, University of North Carolina at Chapel Hill, USA
    • Gene Therapy Center, University of North Carolina at Chapel Hill, USA
    • Corresponding Author InformationCorresponding author. University of North Carolina at Chapel Hill, CB #7236, 1185 1st Floor Physicians Office Building, 170 Manning Drive, Chapel Hill, NC 27599-7236. Tel.: +1 919 966 1178; fax: +1 919 966 0907.
    • ,
  • Junjiang Sun

      Affiliations

    • Gene Therapy Center, University of North Carolina at Chapel Hill, USA

References 

  1. Pawlinski R, Fernandes A, Kehrle B, Pedersen B, Parry G, Erlich J, et al. Tissue factor deficiency causes cardiac fibrosis and left ventricular dysfunction. Proc Natl Acad Sci U S A. Nov 26 2002;99(24):15333–15338
  2. Mackman N. Tissue-specific hemostasis in mice. Arterioscler Thromb Vasc Biol. Nov 2005;25(11):2273–2281
  3. Drake TA, Morrissey JH, Edgington TS. Selective cellular expression of tissue factor in human tissues. Implications for disorders of hemostasis and thrombosis. Am J Pathol. May 1989;134(5):1087–1097
  4. Fleck RA, Rao LV, Rapaport SI, Varki N. Localization of human tissue factor antigen by immunostaining with monospecific, polyclonal anti-human tissue factor antibody. Thromb Res. Jul 15 1990;59(2):421–437
  5. Bernardi F, Dolce A, Pinotti M, Shapiro AD, Santagostino E, Peyvandi F, et al. Major differences in bleeding symptoms between factor VII deficiency and hemophilia B. J Thromb Haemost. May 2009;7(5):774–779
  6. Lapecorella M, Mariani G. Factor VII deficiency: defining the clinical picture and optimizing therapeutic options. Haemophilia. Nov 2008;14(6):1170–1175
  7. Parameswaran R, Shapiro AD, Gill JC, Kessler CM. Dose effect and efficacy of rFVIIa in the treatment of haemophilia patients with inhibitors: analysis from the Hemophilia and Thrombosis Research Society Registry. Haemophilia. Mar 2005;11(2):100–106
  8. Key NS, Aledort LM, Beardsley D, Cooper HA, Davignon G, Ewenstein BM, et al. Home treatment of mild to moderate bleeding episodes using recombinant factor VIIa (Novoseven) in haemophiliacs with inhibitors. Thromb Haemost. Dec 1998;80(6):912–918
  9. Santagostino E, Gringeri A, Mannucci PM. Home treatment with recombinant activated factor VII in patients with factor VIII inhibitors: the advantages of early intervention. Br J Haematol. Jan 1999;104(1):22–26
  10. Monroe DM. Further understanding of recombinant activated factor VII mode of action. Semin Hematol. Apr 2008;45(2 Suppl 1):S7–S11
  11. Weeterings C, Lisman T, de Groot PG. Tissue factor-independent effects of recombinant factor VIIa on hemostasis. Semin Hematol. Apr 2008;45(2 Suppl 1):S12–S15
  12. Valentino LA, Hakobyan N, Rodriguez N, Hoots WK. Pathogenesis of haemophilic synovitis: experimental studies on blood-induced joint damage. Haemophilia. Nov 2007;13(Suppl 3):10–13
  13. Busso N, Morard C, Salvi R, Peclat V, So A. Role of the tissue factor pathway in synovial inflammation. Arthritis Rheum. Mar 2003;48(3):651–659
  14. Berckmans RJ, Nieuwland R, Tak PP, Boing AN, Romijn FP, Kraan MC, et al. Cell-derived microparticles in synovial fluid from inflamed arthritic joints support coagulation exclusively via a factor VII-dependent mechanism. Arthritis Rheum. Nov 2002;46(11):2857–2866
  15. Xue M, March L, Sambrook PN, Fukudome K, Jackson CJ. Endothelial protein C receptor is overexpressed in rheumatoid arthritic (RA) synovium and mediates the anti-inflammatory effects of activated protein C in RA monocytes. Ann Rheum Dis. Dec 2007;66(12):1574–1580
  16. Ronday HK, Smits HH, Van Muijen GN, Pruszczynski MS, Dolhain RJ, Van Langelaan EJ, et al. Difference in expression of the plasminogen activation system in synovial tissue of patients with rheumatoid arthritis and osteoarthritis. Br J Rheumatol. May 1996;35(5):416–423
  17. Agirbasli M, Inanc N, Baykan OA, Direskeneli H. The effects of TNF alpha inhibition on plasma fibrinolytic balance in patients with chronic inflammatory rheumatical disorders. Clin Exp Rheumatol. Sep-Oct 2006;24(5):580–583
  18. Konkle BA, Ebbesen LS, Erhardtsen E, Bianco RP, Lissitchkov T, Rusen L, et al. Randomized, prospective clinical trial of recombinant factor VIIa for secondary prophylaxis in hemophilia patients with inhibitors. J Thromb Haemost. Sep 2007;5(9):1904–1913
  19. Gopalakrishnan R, Hedner U, Ghosh S, Nayak R, Allen TC, Pendurthi UR, et al. Bio-distribution of pharmacologically administered rFVIIa. J Thromb Haemost. Feb 2010;8(2):301–310
  20. Hedner U. Potential role of recombinant factor FVIIa in prophylaxis in severe hemophilia patients with inhibitors. J Thromb Haemost. Nov 2006;4(11):2498–2500
  21. Chang P, Aronson DL, Borenstein DG, Kessler CM. Coagulant proteins and thrombin generation in synovial fluid: a model for extravascular coagulation. Am J Hematol. Oct 1995;50(2):79–83
  22. Fareed J, Callas DD, Hoppensteadt D, Bermes EW. Tissue factor antigen levels in various biological fluids. Blood Coagul Fibrinolysis. Jun 1995;6(Suppl 1):S32–S36
  23. Miller GJ, Howarth DJ, Attfield JC, Cooke CJ, Nanjee MN, Olszewski WL, et al. Haemostatic factors in human peripheral afferent lymph. Thromb Haemost. Mar 2000;83(3):427–432
  24. Valentino LA, Hakobyan N. Histological changes in murine haemophilic synovitis: a quantitative grading system to assess blood-induced synovitis. Haemophilia. Nov 2006;12(6):654–662
  25. Hakobyan N, Enockson C, Cole AA, Rick Sumner D, Valentino LA. Experimental haemophilic arthropathy in a mouse model of a massive haemarthrosis: gross, radiological and histological changes. Haemophilia. Jul 2008;14(4):804–809
  26. Sun J, Hakobyan N, Valentino LA, Feldman BL, Samulski RJ, Monahan PE. Intra-articular factor IX protein or gene replacement protects against development of hemophilic synovitis in the absence of circulating factor IX. Blood. Dec 2008;112(12):4532–4541
  27. Beeby TL, Chasseaud LF, Taylor T, Thomsen MK. Distribution of the recombinant coagulation factor 125I-rFVIIa in rats. Thromb Haemost. Sep 1 1993;70(3):465–468
  28. Lopez-Vilchez K, Tusell J, Hedner U, Altisnet C, Escolar G, Galan AM. Traffic of rFVIIa through endothelial cells and redistribution into subendothelium: Implications for a prolonged hemostatic effect. Journal of Coagulation Disorders. Oct 2009;1(1):1–6
  29. Nayak RC, Sen P, Ghosh S, Gopalakrishnan R, Esmon CT, Pendurthi UR, et al. Endothelial cell protein C receptor cellular localization and trafficking: potential functional implications. Blood. Aug 27 2009;114(9):1974–1986
  30. McDonald A, Hoffman M, Hedner U, Roberts HR, Monroe DM. Restoring hemostatic thrombin generation at the time of cutaneous wounding does not normalize healing in hemophilia B. J Thromb Haemost. Aug 2007;5(8):1577–1583
  31. Aljamali MN, Margaritis P, Schlachterman A, Tai SJ, Roy E, Bunte R, et al. Long-term expression of murine activated factor VII is safe, but elevated levels cause premature mortality. J Clin Invest. May 2008;118(5):1825–1834

PII: S0049-3848(10)00137-4

doi: 10.1016/j.thromres.2010.02.006

Thrombosis Research
Volume 125 , Pages S63-S66 , April 2010

Article Tools