4. Prophylaxis

  1. Christine A. Lee MA, MD, DSc, FRCP, FRCPath, FRCOG2,
  2. Erik E. Berntorp MD, PhD3 and
  3. W. Keith Hoots MD4
  1. Kathelijn Fischer and
  2. H. Marijke van den Berg

Published Online: 24 APR 2014

DOI: 10.1002/9781118398258.ch4

Textbook of Hemophilia, 3

Textbook of Hemophilia, 3

How to Cite

Fischer, K. and van den Berg, H. M. (2014) Prophylaxis, in Textbook of Hemophilia, 3 (eds C. A. Lee, E. E. Berntorp and W. K. Hoots), John Wiley & Sons, Ltd, Oxford, UK. doi: 10.1002/9781118398258.ch4

Editor Information

  1. 2

    Emeritus Professor of Haemophilia, University of London, London, UK

  2. 3

    Professor of Coagulation Medicine, Lund University, Malmö Centre for Thrombosis and Haemostasis, Skåne University Hospital, Malmö, Sweden

  3. 4

    Director, Division of Blood Diseases and Resources, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD; Professor of Pediatrics and Internal Medicine, University of Texas Medical School at Houston, Houston, TX, USA

Author Information

  1. University Medical Centre, Utrecht, the Netherlands

Publication History

  1. Published Online: 24 APR 2014
  2. Published Print: 23 MAY 2014

ISBN Information

Print ISBN: 9781118398241

Online ISBN: 9781118398258



  • Prophylaxis;
  • cost-effectiveness;
  • long-term outcome


Prophylaxis was introduced for severe hemophilia more than 50 years ago in Sweden. Costs of treatment and the need for frequent infusions have delayed the acceptance of this very effective treatment. With primary prophylaxis, bleeding can be prevented and children can lead a normal life. Dosage and frequency of prophylaxis can be individualized to reduce cost and should make prophylaxis affordable for more children. In the Western world, prophylaxis should be the standard of care for all children with severe hemophilia A and B. When to begin prophylaxis in adults with hemophilic arthropathy is still under debate as it is considered less effective.