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Iron chelation therapy in the management of thalassemia: the Asian perspectives

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Abstract

Worldwide, thalassemia is the most commonly inherited hemolytic anemia, and it is most prevalent in Asia and the Middle East. Iron overload represents a significant problem in patients with transfusion-dependent β-thalassemia. Chelation therapy with deferoxamine has traditionally been the standard therapeutic option but its usage is tempered by suboptimal patient compliance due to the discomfort and demands associated with the administration regimen. Therefore, a great deal of attention has been focused on the development of oral chelating agents. Deferiprone, even though available for nearly two decades in Asia with recent encouraging data on cardiac iron removal and long-term efficacy, has serious adverse effects including agranulocytosis and neutropenia which has impeded it from routine clinical practice. A novel oral chelator; deferasirox is effective throughout a 24 h dosing period and both preclinical and clinical data indicate that it successfully removes both hepatic and cardiac iron. In Asia, optimal management of severe thalassemia patients and the availability and access to oral iron chelators still presents a major challenge in many countries. In this regard, the development and implementation of consensus guidelines for management of Asian patients with transfusion-dependent thalassemia will be a major step towards improving and maintaining the continuity of patient care.

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Authors and Affiliations

  1. Siriraj-Thalassemia Research Program, Haematology/Oncology Division, Department of Pediatrics, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, 10700, Thailand

    Vip Viprakasit

  2. Department of Pediatrics, University of Malaya, Kuala lumpur, Malaysia

    Chan Lee-Lee

  3. Hematology/Oncology, Bone Marrow and Cord Blood Transplantation Division, Department of Pediatrics, National University Hospital, Singapore, Singapore

    Quah Thuan Chong

  4. Hematology/Oncology Division, Department of Pediatrics, National Taiwan University Hospital, Taipei, Taiwan

    Kai-Hsin Lin

  5. Siriraj-Thalassemia Research Program, Haematology/Oncology Division, Department of Internal Medicine, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, 10700, Thailand

    Archrob Khuhapinant

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  1. Vip Viprakasit
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  2. Chan Lee-Lee
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  4. Kai-Hsin Lin
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Correspondence to Vip Viprakasit.

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Viprakasit, V., Lee-Lee, C., Chong, Q.T. et al. Iron chelation therapy in the management of thalassemia: the Asian perspectives. Int J Hematol 90, 435–445 (2009). https://doi.org/10.1007/s12185-009-0432-0

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