Iron chelation therapy in the management of thalassemia: the Asian perspectives.
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 beta-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.
Viprakasit
Vip
V
Siriraj-Thalassemia Research Program, Haematology/Oncology Division, Department of Pediatrics, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, 10700, Thailand. sivvp@mahidol.ac.th.
Lee-Lee
Chan
C
Department of Pediatrics, University of Malaya, Kuala lumpur, Malaysia.
Chong
Quah Thuan
QT
Hematology/Oncology, Bone Marrow and Cord Blood Transplantation Division, Department of Pediatrics, National University Hospital, Singapore, Singapore.
Lin
Kai-Hsin
KH
Hematology/Oncology Division, Department of Pediatrics, National Taiwan University Hospital, Taipei, Taiwan.
Khuhapinant
Archrob
A
Siriraj-Thalassemia Research Program, Haematology/Oncology Division, Department of Internal Medicine, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, 10700, Thailand.
D001208:Asia / Q000453:epidemiology; D015913:Chelation Therapy; D003431:Cross-Cultural Comparison; D004333:Drug Administration Routes; D017048:Health Care Costs; D006801:Humans; D007502:Iron Chelating Agents / Q000008:administration & dosage / Q000494:pharmacology / Q000627:therapeutic use*; D019190:Iron Overload / Q000188:drug therapy*; D017410:Practice Guidelines as Topic; D015995:Prevalence; D013789:Thalassemia / Q000191:economics / Q000453:epidemiology / Q000628:therapy*; D065227:Transfusion Reaction; D017086:beta-Thalassemia / Q000191:economics / Q000453:epidemiology / Q000628:therapy
D007502:Iron Chelating Agents