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Published on: September 30, 2021
Prophylaxis in people with haemophilia
Antonio Coppola1, Massimo Franchini, Annarita Tagliaferri
1Regional Reference Centre for Coagulation Disorders, Federico II University Hospital, Naples, Italy. antocopp@unina.it
Thrombosis and Haemostasis
|April 8, 2009
Summary
Primary prophylaxis, regular factor concentrate infusions before age two, is the preferred treatment for severe hemophilia in children. Early intervention prevents joint damage and improves quality of life.
Area of Science:
- Hematology
- Pediatric Medicine
- Orthopedics
Background:
- Severe hemophilia requires lifelong management to prevent bleeding complications.
- Hemophilic arthropathy significantly impacts joint health, psycho-social development, and quality of life.
Purpose of the Study:
- To review current evidence and clinical strategies for prophylactic treatment in severe hemophilia.
- To highlight challenges and open issues in prophylactic regimens for hemophilia patients.
Main Methods:
- Systematic review of randomized controlled studies and four-decade clinical experience.
- Analysis of literature on primary and secondary prophylaxis in severe hemophilia.
Main Results:
- Primary prophylaxis, initiated early, is the first-choice treatment for children with severe hemophilia.
- Early prophylaxis significantly reduces joint damage and improves patient outcomes and quality of life.
- Secondary prophylaxis aims to delay arthropathy progression, with benefits less studied in adolescents and adults.
Conclusions:
- Prophylactic treatment is crucial for managing severe hemophilia, especially when initiated early.
- Further research is needed on optimal regimens, adherence, long-term outcomes, and cost-benefit ratios of prophylaxis.
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