20. Treatment of Bleeding in FVIII Inhibitor Patients

  1. Emérito-Carlos Rodríguez-Merchán MD, PHD3,4 and
  2. Leonard A. Valentino MD5
  1. Paul L. F. Giangrande BSc, MD, FRCP, FRCPath, FRCPCH1 and
  2. Jerome Teitel MD2

Published Online: 12 MAY 2011

DOI: 10.1002/9781119979401.ch20

Current and Future Issues in Hemophilia Care

Current and Future Issues in Hemophilia Care

How to Cite

Giangrande, P. L. F. and Teitel, J. (2011) Treatment of Bleeding in FVIII Inhibitor Patients, in Current and Future Issues in Hemophilia Care (eds E.-C. Rodríguez-Merchán and L. A. Valentino), Wiley-Blackwell, Oxford, UK. doi: 10.1002/9781119979401.ch20

Editor Information

  1. 3

    Department of Orthopedic Surgery and Hemophilia Unit, La Paz University Hospital, Spain

  2. 4

    School of Medicine, Autonomous University, Madrid, Spain

  3. 5

    Hemophilia and Thrombophilia Center, Rush University Medical Center, Chicago, IL, USA

Author Information

  1. 1

    Churchill Hospital, Oxford, UK

  2. 2

    St Michael's Hospital, Toronto, ON, Canada

Publication History

  1. Published Online: 12 MAY 2011
  2. Published Print: 13 MAY 2011

ISBN Information

Print ISBN: 9780470670576

Online ISBN: 9781119979401

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Keywords:

  • activated prothrombin complex concentrates;
  • recombinant activated factor VII;
  • immune tolerance induction;
  • inhibitor bypassing therapy;
  • factor IX inhibitors;
  • surgery;
  • developing countries

Summary

The development of inhibitors usually renders hemophilia A patients refractory to factor VIII replacement therapy. The inhibitor bypassing agents activated PCC and recombinant activated factor VII (rFVIIa) are highly effective for treating bleeding and for providing surgical hemostasis but responses are not entirely predictable, and they cannot be monitored by conventional laboratory assays. Their high cost may make them relatively inaccessible in developing countries. Eradication of inhibitors by induction of immune tolerance (ITI) is achievable in most patients but there is no consensus on optimal regimens. Promising new agents for inhibitor treatment are under development, including recombinant porcine factor VIII and altered rFVIIa molecules with enhanced potency or improved pharmacokinetics. Factor IX inhibitors in hemophilia B patients occur rarely but they are even more problematic, as they may be associated with severe allergic reactions and they respond poorly to ITI.