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Treatment of patients with haemophilia and inhibitory antibodies

  • Symposium on Hematology-I
  • Published:
The Indian Journal of Pediatrics Aims and scope Submit manuscript

Abstract

The development of inhibitory antibodies is a complication which arise in approximately 10% of patients with haemophilia A. The underlying genetic mutation is the single most important predisposing cause, although other risk factors have been identified. Periodic screening for inhibitors is a vital aspect of haemophilia care. The consequences of inhibitor development are very significant in terms of morbidity and cost. Several agents are now available for control of bleeding, but these are often very expensive, the most useful agents include recombinant activated factor VII, prothrombin complex concentrates and porcine factor VIII. It is possible to suppress antibody production with immune tolerance, which is successful in approximately 85% of cases and relapse is rare.

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References

  1. Darby SC, Ewart DW, Giangrande PLF, Dolin PJ, Spooner RJS, Rizza CR. Mortality before and after HIV infection in the complete UK population of haemophiliacs.Nature 1995; 377: 79–82.

    Article  PubMed  CAS  Google Scholar 

  2. Darby SC, Ewart DW, Giangrande PLF, Spooner RJD, Rizza CR. Mortality from liver cancer and liver disease in haemophilic men and boys given blood products contaminated with hepatitisLancet 1997; 350:1425–1431.

    Article  PubMed  CAS  Google Scholar 

  3. Giangrande PLF, Kessler CM, Jenkins CE, Weatherill PJ, Webb PD. Viral pharmacovigilance study of porcine factor VIII.Haemophilia 2002; 8: 798–801.

    Article  PubMed  CAS  Google Scholar 

  4. Giles A, Verbruggen B, Rivard G, Teitel J, Walker. A detailed comparison of the performance of the standard versus the Nijmegen modification of the Bethesda assay in detecting factor VIII:C inhibitors in the haemophilia A population of Canada.Thrombosis and Haemostasis; 1998; 79: 872–876.

    PubMed  CAS  Google Scholar 

  5. Gringeri A, Santagostino E, Tradati F, Giangrande PLF, Mannucci PM. Adverse effects of treatment with porcine factor VIII.Thrombosis and Haemostasis 1991; 65: 245–247.

    PubMed  CAS  Google Scholar 

  6. Hay CR, Baglin TP, Collins PW, Hill FG, Keeling DM. The diagnosis and management of factor VIII and IX inhibitors: a guideline from the United kingdom Haemophilia Centre Doctors’ Organisation (UKHCDO).Brit J Haemat 2000; 111: 78–90.

    Article  PubMed  CAS  Google Scholar 

  7. Key NS on behalf of the US rVIIa Home Therapy Study Group. Home treatment of mild to moderate bleeding episodes using recombinant factor VIIa (Novseven) in haemophiliacs with inhibitors.Thrombosis and Haemostasis 1998; 80:912–918.

    PubMed  CAS  Google Scholar 

  8. Lacroix-Desmazes S, Bayry J, Misra N, Horn MP, Villard S, Pashov Aet al. The prevalence of proteolytic antibodies against factor VIII in hemophilia A.N Eng J Med 2002; 346: 662–667.

    Article  CAS  Google Scholar 

  9. Linden JV, Kolakoski MH, Lima JE, Du JE, Lipton RA. Factor concentrate usage in persons with hemophilia in New York State.Transfusion 2003; 43: 470–475.

    Article  PubMed  Google Scholar 

  10. Lorenzo JI, Lopez A, Altisent C, Aznar JA. Incidence of factor Vinhibitors in severe haemophilia: the importance of patient age.Brit J Haem 2001; 113:600–603.

    Article  CAS  Google Scholar 

  11. Lusher JM, Shapiro SS, Palascak JE, Rao AV, Levine PH, Blatt M. Efficacy of prothrombin-complex concentrates in hemophiliacs with antibodies for factor VIII. A multicenter trial.New Eng J Med 1980; 303: 421–425.

    Article  PubMed  CAS  Google Scholar 

  12. Lusher JM, Ingerslev J, Roberts H, Hedner U. Clinical experience with recombinant factor Vila.Blood Coagulation and Fibrinolysis 1998; 9:119–128.

    Article  PubMed  CAS  Google Scholar 

  13. Mariani G, Siragusa S, Kroner BL.Seminars in Thrombosis and Haemostasis 2003; 29:69–76.

    Article  CAS  Google Scholar 

  14. Mauser-Bunschoten EP, van der Bom JG, Bongers M, Twijnstra M, Roosendaal G, Fischeret al. Purity of factor VIII product and incidence of inhibitors in previously untreated patients with haemophilia A.Haemophilia 2001; 7:364–368.

    Article  PubMed  CAS  Google Scholar 

  15. Oldenburg J, El-Maari O, Schwaab R. Inhibitor development in correlation to factor VIII genotypes.Haemophilia 2002; 8 (Suppl. 2): 23–29.

    Article  PubMed  CAS  Google Scholar 

  16. Rizza CR, Spooner RJD. Treatment of haemophilia and related disorders in Britain and Northern Ireland during 1976–1980: report on behalf of the directors of haemophilia centres in the United Kingdom.Brit Med Journal 1983; 286:929–933.

    Article  CAS  Google Scholar 

  17. Rizza CR, Spooner RJD, Giangrande PLF on behalf of the UK Haemophilia Centre Doctors’ Organisation (UKHCDO) Treatment of haemophilia in the United Kingdom.Haemophilia 1981–1996; 2001; 7:349–359.

    Article  CAS  Google Scholar 

  18. Roberts HR. Inhibitors and their management. In Rizza CR and Lowe G, eds. Management of Haemophilia & Other Bleeding Disorders. (WB Saunders 1997)

  19. Saenko EL, Ananyeva NM, Kouiavskala DV, Khrenov AV, Anderson JA, Shima M, Qian J, Scott D. Haemophilia A: effect of inhibitory antibodies on factor VIII functional interactions and approaches to prevent their action.Haemophilia 2002 ; 8:1–11.

    Article  PubMed  CAS  Google Scholar 

  20. Santagostino E, Gringeri A, Mannucci P.M. Home treatment with recombinant activated factor VII in patients with factor VIII inhibitors: the advantages of early treatment.Brit J Haem 1999; 104:22–26.

    Article  CAS  Google Scholar 

  21. Scharrer I, Bray GL, Neutzling O. Incidence of inhibitors in haemophilia A patients-a review of recent studies of recombinant and plasma-derived factor VIII concentrates.Haemophilia 1999; 5:145–154.

    Article  PubMed  CAS  Google Scholar 

  22. Sjamsoedin LJ, Heijnen L, Mauser-Bunschoten EP, van Geijswijk JL, van Houwelingen H. van Asten P, Sixma J.J. The effect of activated prothrombin-complex concentrate (FEIBA) on joint and muscle bleeding in patients with haemophilia A and antibodies to factor VIII. A double-blind clinical trial.N Eng J Med 1981; 305:717–721.

    Article  CAS  Google Scholar 

  23. Verbruggen B, Novakova RR. Wessels H. Boezman I, van den Berg M, Mauser-Bunschoten E. The Nijmegen modification of the Bethesda assay for factor VIII:C inhibitors: improved specificity and reliability.Thrombosis and Haemostasis 1995; 73: 247–250.

    PubMed  CAS  Google Scholar 

  24. Warrier I. Management of haemophilia patients with inhibitors and anaphylaxis.Haemophilia 1998a; 4:574–576.

    Article  PubMed  CAS  Google Scholar 

  25. Warrier I, Lenk H, Saldi. P, Pollman H, Tengborn L, Berntorp, E. Nephrotic syndrome in hemophilia B patients with inhibitors.Haemophilia 1998b; 4:248–251.

    Article  Google Scholar 

  26. Wight J, Richards M. Very high cost treatment for a single individual-a case report.J Public Health Medicine 2003; 25:4–7.

    Article  Google Scholar 

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Authors and Affiliations

  1. Oxford Haemophilia Centre and Thrombosis Unit, Churchill Hospital, OX37LJ, Oxford, UK

    P. L. F. Giangrande

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  1. P. L. F. Giangrande
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Correspondence to P. L. F. Giangrande.

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Giangrande, P.L.F. Treatment of patients with haemophilia and inhibitory antibodies. Indian J Pediatr 70, 655–659 (2003). https://doi.org/10.1007/BF02724256

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