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Continuous Manual Exchange Transfusion for Patients with Sickle Cell Disease: An Efficient Method to Avoid Iron Overload
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Evaluation and treatment of transfusional iron overload in children

Hannah M Ware1, Janet L Kwiatkowski

  • 1Division of Hematology, Department of Pediatrics, Children's Hospital of Philadelphia, University of Pennsylvania, 34th Street, Civic Center Boulevard, Philadelphia, PA 19104, USA.

Pediatric Clinics of North America
|November 19, 2013
PubMed

Insights

Regular blood transfusions for anemias like thalassemia can cause iron overload. Iron chelation therapy is crucial for removing excess iron and preventing organ damage in children. Treatment choice depends on overload severity and patient factors.

Area of Science:

  • Hematology
  • Pediatric Medicine
  • Pharmacology

Background:

  • Red blood cell transfusions are vital for managing anemias such as thalassemia and sickle cell disease.
  • Transfusional iron overload is a significant complication in children requiring regular transfusions due to the body's inability to excrete excess iron.
  • Iron overload can lead to severe damage in critical organs including the heart, liver, and endocrine system.

Purpose of the Study:

  • To review the current landscape of iron chelation therapy for managing transfusional iron overload in pediatric patients.
  • To outline the available iron chelators and factors influencing treatment selection.

Main Methods:

  • Review of existing literature on iron chelation therapy in pediatric anemia management.
  • Analysis of the characteristics and clinical considerations for available iron chelators: deferoxamine, deferasirox, and deferiprone.

Main Results:

  • Three primary iron chelators are available in the United States: deferoxamine, deferasirox, and deferiprone.
  • Patient management requires careful consideration of the severity of iron overload, the chosen chelator's administration schedule, and its specific adverse effect profile.

Conclusions:

  • Iron chelation therapy is essential for mitigating the risks associated with transfusional iron overload in children with chronic anemias.
  • Treatment decisions should be individualized, balancing efficacy, safety, and patient-specific factors to optimize outcomes and prevent long-term organ damage.

Keywords:
ChelationIron overloadMagnetic resonance imagingTransfusion

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