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Iron Chelator Drugs in Transfusion Dependent Thalassemia: a Review
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M Hashemieh    |
| Department of Pediatric Blood and Oncology, Shahid Beheshti University of Medical Sciences, Tehran, Iran , mozhganhashemieh@gmail.com |
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Abstract: (7100 Views) |
Background & aim: Thalassemia syndromes are the most common inherited hemoglobinopathies in the world characterized by various degrees of defective production of the alpha or beta globin chains. Iran is one of the countries located on the thalassemia belt and therefore thalassemia syndromes have a significant importance in our country. The most important mainstay of treatment in beta thalassemia is packed red blood cell transfusion. Thalassemic patients need lifelong transfusions and the consequence of these repeated transfusions is iron accumulation in different organs, such as heart, liver and endocrine gland.
Methods: In this review, an electronic search was performed in databases of PubMed, Google Scholar, Scopus and Science Direct within English literature (from January 2000 to January 2020). In this search 148 articles were found. The title, abstract and full text of all documents identified and those describing iron chelator drugs in thalassemic patients were finally selected. Overall, 124 of which were excluded because they did not correlate with the main topic or were duplicate.
Results: The oldest iron chelator drug is deferoxamine which was administered via subcutaneous or intravenous injection. The first oral iron chelator is deferiprone and the second drug, deferasirox. In recent years, deferasirox coated tablets with the brand name Jadenu have been launched, which no longer need to be dissolved in water and has gained special popularity among thalassemia patients.
Conclusion: Iron overload due to repeated transfusions results in organ dysfunction and finally heart failure, liver cirrhosis and multiple endocrinopathies develops. In order to prevent iron accumulation in vital organs, consumption of iron chelator drugs is necessary. Without the use of these drugs, the survival of thalassemia patients will be significantly reduced. Therefore, familiarity with iron chelator drugs has particular importance.
Keywords: Thalassemia, Iron overload, Iron chelation, Deferoxamine, Deferiprone, Deferasirox
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| Keywords: Thalassemia, Iron overload, Iron chelation, Deferoxamine, Deferiprone, Deferasirox |
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Full-Text [PDF 1943 kb]
(612 Downloads)
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Type of Study: Review Article |
Subject:
Clinical
Received: 2020/02/9 | Accepted: 2020/07/12 | Published: 2020/09/5
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