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Tertiary prophylaxis with extended half-life factor prophylaxis: a model to reduce disability in low- and
Archit S Pandharipande1, Silky Jain1, Anuj Singh1
1Department of Pediatric Hematology Oncology, Post Graduate Institute of Child Health, Noida, Uttar Pradesh, 201303, India.
Introduction:
The real-world data on treatment and outcome of hemophilia patients using extended half-life products in developing countries remain scarce. This is largely due to delayed diagnosis, poor joint outcomes, increased morbidity, and limited access to prophylaxis and newer products for treatment and prevention.
Aim:
To analyze the response to extended half-life factor (EHL) prophylaxis in patients with severe hemophilia A and B with advanced arthropathy.
Methods:
Patients with severe hemophilia A and B who received EHL factor concentrates for prophylaxis at our center were included in this analysis. Data collected included bleed frequency, joint involvement, annualized bleed rate (ABR), number of hospital visits, and Hemophilia Joint Health Score (HJHS) prior to prophylaxis. Breakthrough bleeds while on prophylaxis were also recorded.
Results:
A total of 31 patients were started on EHL prophylaxis and followed up for a period ranging from 4 to 91 weeks. A reduction in the bleeding rate was noticed in all with significant reversal of target joints. Additionally, patients remained bleed-free during rehabilitation following joint surgery as well as psoas bleed-related compression neuropathy.
Conclusion:
EHL prophylaxis appears to be an effective strategy even for patients with baseline target joints with significant arthropathy, thus reducing the extent of disability in these patients.
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