An Evidence-Based Approach to Treatment with Iron Chelators in Transfusion- Dependent Thalassemia Patients : Present Trends and Future Scenario

- Authors: Tirin Babu1, George Mathew Panachiyil2
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View Affiliations Hide AffiliationsAffiliations: 1 College of Medicine, Nursing and Health Sciences, National University of Ireland, Galway,Ireland 2 College of Medicine, Nursing and Health Sciences, National University of Ireland, Galway,Ireland
- Source: Frontiers in Clinical Drug Research - Hematology: Volume 5 , pp 50-89
- Publication Date: March 2022
- Language: English
Treatment of hemochromatosis is a significant target-based care of transfusion-dependent thalassemia and non-transfusion dependent thalassemia patients. In some cases hemochromatosis is a secondary concern to frequent red blood cell transfusions as in transfusion-dependent thalassemia (TDT) or advances from enhanced gastrointestinal iron absorption such as in non-transfusion dependent thalassemia (NTDT), this can cause serious illness and death to the patients. When thalassemia major patients undergo frequent blood transfusions, hemochromatosis is unavoidable because the human body lacks a physiological mechanism to evacuate extra iron. Thalassemia patients with transfusional hemochromatosis regularly need treatment with iron chelators to decline the iron overload and thereby retard long-term effects related to iron accumulation in tissues. Deferoxamine, deferiprone, and deferasirox are the three currently approved iron chelators for the treatment of hemochromatosis in transfusion-dependent thalassemia patients. Today, iron chelation therapy's goal is to sustain acceptable levels of iron in the human body at all times. Correct tailoring with iron chelators and their dose modifications must implement on time. We first describe the pathophysiology of hemochromatosis in thalassemia patients in this chapter. We then cover iron chelation therapy's general goals, the features of the permitted iron chelators, and the evidence-based practice behind the usage of iron chelators as a single drug, or as part of combination therapy, and the mechanisms by which chelators work. The guiding principles for monitoring treatment with iron chelators to reduce the toxicity risks from iron chelation are later explained. Finally, the importance of deferasirox twice-daily dose instead of a once-daily dose in transfusiondependent thalassemia patients with inadequate response to high doses and the future directions in treating iron overload in thalassemia patients is discussed.
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