Management of hemophilia in patients with inhibitors: the perspective from developing countries

Semin Thromb Hemost. 2009 Nov;35(8):820-6. doi: 10.1055/s-0029-1245115. Epub 2010 Feb 18.

Abstract

Data are limited on inhibitors in people with hemophilia (PWH) in developing countries. There is a perception that the overall prevalence of inhibitors, ranging from 7 to 19% in different reports, may be lower in these countries as compared with that reported from developed countries. This is possible given the fact that most patients are treated after 2 years of age with plasma-derived clotting factor concentrates. Whether genetic or other environmental factors also contribute to this needs further evaluation. There is a need to develop laboratory infrastructure and establish quality control programs for laboratory tests for inhibitors in developing countries. Management options vary widely given the socioeconomic diversity among these countries. Significant individualization of approach to management is therefore required depending on the available resources, particularly with regard to the use of bypassing agents. The limited data on immune tolerance induction with some low-dose regimens deserve further evaluation. Even in resource-constrained environments, education and a policy of systematic screening of patients associated with judicious use of bypassing agents can significantly improve the care of PWH who develop inhibitors.

Publication types

  • Review

MeSH terms

  • Black People
  • Blood Coagulation Factors / administration & dosage
  • Blood Coagulation Factors / economics
  • Blood Coagulation Factors / therapeutic use
  • Developing Countries
  • Factor VIII / genetics
  • Factor VIII / immunology
  • Factor VIII / therapeutic use
  • Factor VIIa / therapeutic use
  • Hemophilia A / economics
  • Hemophilia A / therapy*
  • Hemorrhage / drug therapy
  • Humans
  • Polymorphism, Genetic
  • Prevalence
  • Recombinant Proteins / therapeutic use

Substances

  • Blood Coagulation Factors
  • Recombinant Proteins
  • Factor VIII
  • recombinant FVIIa
  • anti-inhibitor coagulant complex
  • Factor VIIa