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Management of hemophilia in patients with inhibitors: the perspective from developing countries
Vikram Mathews1, Sukesh C Nair, Sachin David
1Department of Hematology, Christian Medical College, Vellore, India.
Insights
Data on inhibitors in people with hemophilia (PWH) in developing countries are scarce. Improved laboratory infrastructure, screening, and tailored management strategies are crucial for better patient care.
Area of Science:
- Hematology
- Immunology
- Global Health
Background:
- Limited data exist on inhibitor prevalence in people with hemophilia (PWH) in developing nations.
- A perception suggests lower inhibitor rates compared to developed countries, possibly due to delayed treatment with plasma-derived clotting factors.
- Genetic and environmental factors require further investigation.
Purpose of the Study:
- To highlight the limited data on inhibitors in PWH in developing countries.
- To emphasize the need for improved laboratory infrastructure and quality control for inhibitor testing.
- To discuss individualized management strategies considering socioeconomic diversity and resource availability.
Main Methods:
- Review of existing literature and data on inhibitor prevalence in PWH.
- Analysis of treatment practices and their potential impact on inhibitor development.
- Discussion of management options, including bypassing agents and immune tolerance induction.
Main Results:
- Inhibitor prevalence data in developing countries are scarce, with estimates ranging from 7-19%.
- Treatment initiation after two years of age with plasma-derived concentrates may influence inhibitor rates.
- Management approaches must be individualized due to significant socioeconomic variations.
Conclusions:
- Developing countries require enhanced laboratory infrastructure and quality control for inhibitor diagnostics.
- Judicious use of bypassing agents and systematic patient screening can improve care for PWH with inhibitors.
- Further research on immune tolerance induction with low-dose regimens is warranted.
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.
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