17. Non-Genetic Risk Factors for Inhibitor Development

  1. Emérito-Carlos Rodríguez-Merchán MD, PHD2,3 and
  2. Leonard A. Valentino MD4
  1. Lisa N. Boggio MS, MD and
  2. Mindy L. Simpson MD

Published Online: 12 MAY 2011

DOI: 10.1002/9781119979401.ch17

Current and Future Issues in Hemophilia Care

Current and Future Issues in Hemophilia Care

How to Cite

Boggio, L. N. and Simpson, M. L. (2011) Non-Genetic Risk Factors for Inhibitor Development, 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.ch17

Editor Information

  1. 2

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

  2. 3

    School of Medicine, Autonomous University, Madrid, Spain

  3. 4

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

Author Information

  1. Rush University Medical Center, Chicago, IL, USA

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:

  • hemophilia;
  • Inhibitor;
  • environmental risk;
  • factor treatment;
  • immune challenge;
  • pregnancy

Summary

The most serious complication of hemophilia is the development of inhibitors. Patients with inhibitors to factor VIII or IX have bleeding which may not be responsive to traditional factor replacement and is therefore more difficult to control. Inhibitors develop in 20–30% of patients with severe hemophilia A (factor VIII deficiency) and up to 5% of those with severe hemophilia B (factor IX deficiency) patients. There are genetic and non-genetic risk factors related to inhibitor formation. Non-genetic risk factors may include factor therapy, immune system challenges, and pregnancy or neonatal periods. Several research studies have attempted to evaluate the contribution of each risk factor, but larger studies are still needed.