Iron Chelation & Overload
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Scientific Literature
Iron Chelation Therapy for Children with Transfusion‐Dependent β‐Thalassemia: How Young Is Too Young?
In this review, we provide a summary of evidence on iron overload in young children with transfusion-dependent β-thalassemia (TDT) and explore the ideal timing for intervention. Key data from clinical…
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Scientific Literature
Revisiting Iron Overload Status and Change Thresholds as Predictors of Mortality in Transfusion-Dependent β-Thalassemia: A 10-year Cohort Study
Data on iron overload status and change thresholds that can predict mortality in patients with transfusion-dependent β-thalassemia (TDT) are limited. This was a retrospective cohort study of 912 TDT patients…
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Scientific Literature
Deferiprone vs Deferoxamine for Transfusional Iron Overload in Sickle Cell Disease and Other Anemias: Pediatric Subgroup Analysis of the Randomized, Open-label FIRST Study
Background: Children with sickle cell disease (SCD) who are chronically transfused often, require iron chelation therapy. There are limited data that allow for comparison of the efficacy and safety of…
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Scientific Literature
Efficacy and Safety of Early-start Deferiprone in Infants and Young Children with Transfusion-dependent Beta Thalassemia: Evidence for Iron Shuttling to Transferrin in a Randomized, Double-blind, Placebo-controlled, Clinical Trial (START)
Children with transfusion‐dependent thalassemia (TDT) require regular blood transfusions that, without iron‐chelation therapy, lead to iron‐overload toxicities. Current practice delays chelation therapy (late‐start) until reaching iron overload (serum ferritin ≥1000 μg/L)…
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Scientific Literature
Deferiprone for Transfusional Iron Overload in Sickle Cell Disease and Other Anemias: Open-label Study of Up to 3 Years
Long-term safety and efficacy data on the iron chelator deferiprone in sickle cell disease (SCD) and other anemias are limited. FIRST-EXT was a 2-year extension study of FIRST (Ferriprox in…
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Scientific Literature
Promoting Adherence to Iron Chelation Treatment in Beta-Thalassemia Patients
Thalassaemia is one of the commonest inherited genetic disorders world-wide with around 25,000 births of the most severely affected transfusion dependent children annually. Patients with transfusion dependent thalassaemia require regular…
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Scientific Literature
Guidelines for the Monitoring and Management of Iron Overload in Patients with Haemoglobinopathies and Rare Anaemias
These Guidelines were compiled according to the BSH process at https://b-s-h.org.uk/guidelines/proposing-and-writing-a-new-bsh-guideline/. The Grading of Recommendations Assessment, Development and Evaluation (GRADE) nomenclaturewas used to evaluate levels of evidence and to assess…
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Scientific Literature
Iron Metabolism Under Conditions of Ineffective Erythropoiesis in β-Thalassemia
β-Thalassemia (BT) is an inherited genetic disorder that is characterized by ineffective erythropoiesis (IE), leading to anemia and abnormal iron metabolism. IE is an abnormal expansion of the number of…
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Scientific Literature
Iron Chelators or Therapeutic Modulators of Iron Overload: Are We Anywhere Near Ideal One?
Iron overload is an important cause of mortality and morbidity in chronic transfusion-dependent states commonly exemplified by transfusion-dependent beta-thalassaemia syndromes [including non-transfusion-dependent (NTD) thalassaemia intermedia] as well as transfusion-dependent myelodysplastic…
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