title: Iron-chelation therapy with oral chelators in patients with thalassemia major.
abstract: In thalassemia major (TM), without iron chelation therapy, iron-mediated free radical damage causes liver, endocrine, and myocardial toxicities. Deferoxamine has universally been the standard therapeutic option for iron chelation therapy; however, its usage is troublesome, leading to  suboptimal patient compliance. In order to maximize the effectiveness of iron chelation therapy, oral iron chelators deferiprone and deferasirox constitute an important development, offering a potential to improve compliance. Although both oral drugs are effective, they have differences including  different pharmacokinetics and side-effect profiles. Our retrospective evaluation of TM patients using oral chelators showed that oral chelators are effective in reducing iron overload regarding ferritin level and partially in cardiac T2* value. However, in our study side effects and discontinuation  rates were unexpectedly high and close follow-up of TM patients using oral chelators should be carefully done. The variability in rate of side effects and drug discontinuation in spelenectomized patients using oral chelators suggests that spleen may have a role in pharmacokinetics of these  drugs, as well.
authors: Antalya Egitim ve Arastirma Hastanesi Çocuk Hematoloji Bolumu, 07100 Antalya, Turkey. vedat@akdeniz.edu.tr
authors: Vedat
authors: V
authors: Uygun
authors: Erdal
authors: E
authors: Kurtoglu
mesh_terms: D000293:Adolescent; D000328:Adult; D001565:Benzoates / Q000009:adverse effects / Q000627:therapeutic use; D001803:Blood Transfusion; D015913:Chelation Therapy / Q000009:adverse effects / Q000379:methods; D002648:Child; D002675:Child, Preschool; D000077588:Deferasirox; D000077543:Deferiprone; D003676:Deferoxamine / Q000009:adverse effects / Q000627:therapeutic use; D006801:Humans; D007502:Iron Chelating Agents / Q000009:adverse effects / Q000627:therapeutic use*; D008875:Middle Aged; D011728:Pyridones / Q000009:adverse effects / Q000627:therapeutic use; D012189:Retrospective Studies; D013156:Splenectomy; D014230:Triazoles / Q000009:adverse effects / Q000627:therapeutic use; D055815:Young Adult; D017086:beta-Thalassemia / Q000188:drug therapy* / Q000628:therapy
chemical_list: D001565:Benzoates; D007502:Iron Chelating Agents; D011728:Pyridones; D014230:Triazoles; D000077543:Deferiprone; D003676:Deferoxamine; D000077588:Deferasirox