Seminars in Hematology
Volume 43 , Pages S42-S47 , January 2006

Steps Towards an Effective Treatment Strategy in Congenital Factor VII Deficiency

  • Guglielmo Mariani

      Affiliations

    • L’Aquila University Medical School, Department of Medicine and Public Health, L’Aquila, Italy.
    • Corresponding Author InformationAddress correspondence to Guglielmo Mariani, Full Professor of Hematology, Universita degli Studi de L’Aquila, Ematologia Dipartimento di Medicina Internae Sanita Pubblica, Largo Tommasi 1, Coppito, 67100 L’Aquila, Italy.
    • ,
  • Mario Lapecorella

      Affiliations

    • Hematology Unit, Ospedale S. Salvatore, L’Aquila, Italy.
    • ,
  • Alberto Dolce

      Affiliations

    • Department of Economic Development, Sicily Region, Palermo, Italy.

References 

  1. Mariani G , Dolce A . Congenital factor VII deficiency . In:  Lee C ,  Berntorp E ,  Hoots K editor. Textbook of Hemophilia . Malden, MA: Blackwell; 2005;p. 311
  2. Peyvandi F , Mannucci PM , Asti D , Abdoullahi M , Di Rocco N , Sharifian R . Clinical manifestations in 28 Italian and Iranian patients with severe factor VII deficiency . Haemophilia . 1997;3:242–246
  3. Ragni MV , Lewis JH , Spero JA , Hasiba U . Factor VII deficiency . Am J Hematol . 1981;10:79–88
  4. Mariani G , Mazzucconi MG . Factor VII congenital deficiency. Clinical picture and classification of the variants . Haemostasis . 1983;13:169–177
  5. Mariani G , Herrmann F , Dolce A , Batorova A , Etro D , Peyvandi F , et al.  Clinical phenotypes and factor VII genotype in congenital factor VII deficiency . Thromb Haemost . 2005;93:481–487
  6. Giansily-Blaizot M , Biron-Andreani C , Aguilar-Martinez P , de Moeloose P , Briquel ME , Goudemand J , et al.  Inherited factor VII deficiency and surgery (Clinical data are the best criteria to predict the risk of bleeding) . Br J Haematol . 2002;117:172–175
  7. Mariani G , Dolce A , Marchetti G , Bernardi F . Clinical picture and management of congenital factor VII deficiency . Haemophilia . 2004;10(suppl 4):180–183
  8. Mariani G , Mannucci PM , Mazzucconi MG , Capitanio A . Treatment of congenital factor VII deficiency with a new concentrate . Thromb Haemost . 1978;39:675–682
  9. Berrettini M , Mariani G , Schiavoni M , Rocino A , Di Paolantonio T , Longo G , et al.  Pharmacokinetic evaluation of recombinant, activated factor VII in patients with inherited factor deficiency . Haematologica . 2001;86:640–645
  10. Erhardtsen E . Pharmacokinetics of recombinant activated factor VII (rFVIIa) . Semin Thromb Hemost . 2000;26:385–391
  11. Villar A , Aronis S , Morfini M , Santagostino E , Auerswald G , Thomsen HF , et al.  Pharmacokinetics of activated recombinant coagulation factor VII (NovoSeven) in children vs. adults with haemophilia A . Haemophilia . 2004;10:352–359
  12. Ingerslev J , Kristensen HL . Clinical picture and treatment strategies in factor VII deficiency . Haemophilia . 1998;4:689–696
  13. Mariani G , Herrmann FH , Schulman S , Batorova A , Wulff K , Etro D , et al.  Thrombosis in inherited factor VII deficiency . J Thromb Haemost . 2003;1:2153–2158
  14. Eskandari N , Feldman N , Greenspoon JS . Factor VII deficiency in pregnancy treated with recombinant factor VIIa . Obstet Gynecol . 2002;99:935–937
  15. Perry DJ . Factor VII deficiency . Br J Haematol . 2002;118:689–700
  16. United Kingdom Haemophilia Centre Doctors’ Organisation (UKHCDO) . Guidelines on the selection and use of therapeutic products to treat haemophilia and other hereditary bleeding disorders . Haemophilia . 2003;9:1–23
  17. Mariani G, Konkle BA, Ingerslev J: Congenital factor VII deficiency: Therapy with recombinant activated factor VII. A critical appraisal. Haemophilia (in press)
  18. Berry EW , Teague L , Pitcher L , Ockelford P . NovoSeven® prophylaxis for severe FVII deficiency . 2001; Abstract from the Free-Paper Session and the Poster Session presented at the 6th Novo Nordisk Symposium on Treatment of Bleeding and Thrombotic Disorders, Copenhagen, Denmark, May 3-4
  19. Mathijssen NCJ , Masereeuw R , Verbeek K , Lavergne JM , Costa J-M , van Heerde WL , et al.  Prophylactic effect of recombinant factor VIIa in factor VII deficient patients . Br J Haematol . 2004;125:494–499
  20. Muleo G , Santoro R , Iannaccaro P , Papaleo G , Elia L , Leo F , et al.   rFVIIa in congenital FVII deficiency . Thromb Res . 1998;91(suppl 1):S86; (abstr 169)
  21. Mariani G , Testa MG , Di Paolantonio T , Molskov Bech R , Hedner U . Use of recombinant, activated factor VII in the treatment of congenital factor VII deficiencies . Vox Sang . 1999;77:131–136
  22. Brummel Ziedins K , Rivard GE , Pouliot RL , Butenas S , Gissel M , Parhami-Seren B , et al.  Factor VIIa replacement therapy in factor VII deficiency . J Thromb Haemost . 2004;2:1735–1744
  23. Aledort LM . Comparative thrombotic event incidence after infusion of recombinant factor VIIa versus factor VIII bypass activity . J Thromb Haemost . 2004;2:1700–1708
  24. Hay CRM . Thrombosis and recombinant factor VIIa (commentary) . J Thromb Haemost . 2004;2:1698–1699
  25. Ingerslev J , Christiansen K , Sørensen B . Inhibitors to factor VII in severe factor VII deficiency (Detection and course of inhibitory response) . J Thromb Haemost . 2005;3:799–800
  26. Bergmann F , Scharrer I . Transient factor VII inhibitor after replacement therapy with recombinant FVIIa (rFVIIa) in a child with FVII deficiency . Bone Marrow Transplant . 1998;92(suppl 1):104; (abstr 3430)
  27. Nicolaisen EM . Antigenicity of activated recombinant factor VII followed through nine years of clinical experience . Blood Coagul Fibrinolysis . 1998;9(suppl 1):S119–S123

PII: S0037-1963(05)00224-6

doi: 10.1053/j.seminhematol.2005.11.017

Seminars in Hematology
Volume 43 , Pages S42-S47 , January 2006

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