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Year : 2011  |  Volume : 2  |  Issue : 4  |  Page : 291-293

Four Decades of Chelation Therapy for β-Thalassemia Major

Ain Shams University, ESPHO President & ICIS Board Member, Cairo, Egypt

Correspondence Address:
Mohsen Saleh Elalfy
Ain Shams University, ESPHO President & ICIS Board Member, Cairo
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Source of Support: None, Conflict of Interest: None

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After discussing the need for iron-chelating therapy with b-thalassemia major patients and their families, we describe the advantages and disadvantages of available chelators-subcutaneous desferrioxamine (DFO), oral deferiprone (DFP), and deferasirox (DFX)-so that a genuinely informed decision can be made. At present, the most frequent choices are either oral DFP or DFX. patient information about the number of previous transfusions and the rate of ongoing transfusion and arrange for hepatic transverse relaxation rate measurements, magnetic resonance imaging evaluation of cardiac T2*, and echocardiographic and electrocardiographic studies. Auditory and ophthalmic testing, including slit-lamp and fundus examinations, are also performed. Laboratory tests include a complete blood count with a differential count and measurement of serum creatinine, protein/creatinine ratio, serum aminotransferases, bilirubin levels, and iron indexes. After transfusing 10-12 units of blood, or reaching a hepatic iron concentration between 3 and 7 mg/g, we prescribe either once-daily oral DFX therapy (20 mg/kg) or DFP (75 mg/kg/d) in escalating doses. Monitoring efficacy and adverse events is valuable. Ideally, iron-chelating therapy should be initiated on a prophylactic basis before clinically significant iron accumulation has occurred. Patients who have already undergone repeated transfusion without sufficient chelation can also be treated successfully.

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