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[Long-term blood transfusion in sickle-cell anemia]
Archives Francaises De Pediatrie
|May 1, 1987
Summary
Periodic red blood cell transfusions offer clinical benefits for pediatric sickle cell disease patients, including reduced pain crises and improved growth. However, iron overload and HIV contamination are significant risks requiring management.
Area of Science:
- Hematology
- Pediatric Medicine
- Transfusion Medicine
Context:
- Sickle cell disease (SCD) is a genetic blood disorder requiring chronic management.
- Homozygous SCD patients often face severe complications necessitating interventions like blood transfusions.
- Transfusion programs aim to mitigate SCD-related morbidity in pediatric populations.
Purpose:
- To evaluate the clinical efficacy and safety of periodic red blood cell transfusions in children and adolescents with homozygous sickle cell disease.
- To assess the impact of transfusions on various SCD complications, including stroke, painful crises, anemia, and organ-specific issues.
- To monitor for adverse events associated with long-term transfusion therapy, such as iron overload and alloimmunization.
Summary:
- Ten patients with homozygous sickle cell disease (ages 4-20) received regular red blood cell transfusions for indications including stroke, painful crises, chronic anemia, and organ damage.
- Transfusions lasted 6 months to 6 years, with phlebotomy used in 7 cases to manage iron overload.
- Clinical benefits observed included decreased painful crises, healing of leg ulcers, improved growth, and pubertal development. However, benefits were less clear for stroke and other conditions.
- No red cell antibodies developed, but 3 children experienced HIV contamination.
- Iron overload occurred in all patients receiving transfusions for over a year, with 2 requiring deferoxamine chelation therapy.
Impact:
- Periodic red blood cell transfusions can provide significant clinical benefits for pediatric homozygous sickle cell disease patients, improving quality of life and addressing specific complications.
- Long-term transfusion therapy necessitates vigilant monitoring and management of potential adverse effects, primarily iron overload and infectious risks.
- This study highlights the complex risk-benefit balance of transfusion programs in managing severe pediatric sickle cell disease.