Al-Kuraishy Hayder M, Al-Gareeb Ali I
Department of Pharmacology, Toxicology and Medicine, College of Medicine, Al-Mustansiriya University, Baghdad, Iraq.
Asian J Transfus Sci. 2017 Jan-Jun;11(1):13-17. doi: 10.4103/0973-6247.200768.
Beta-thalassemias are a cluster of inherited (autosomal recessive) hematological disorders prevalent in the Mediterranean area due to defects in synthesis of β chains of hemoglobin. The aim of present study was to compare the effects of deferasirox and deferoxamine on iron overload and immunological changes in patients with blood transfusion-dependent β-thalassemia major and intermedia.
This study involved 64 patients with known cases of β-thalassemia major or intermedia that has been treated with blood transfusion and iron chelators. Serum ferritin, serum iron, serum total iron binding, unsaturated iron-binding capacity (UIBC), and immunological parameters were assessed in deferoxamine and deferasirox-treated patients.
In deferoxamine-treated patients, serum ferritin levels were high (8160.33 ± 233.75 ng/dL) compared to deferasirox-treated patients (3000.62 ± 188.23 ng/dL; < 0.0001), also there were significant differences in serum iron, total iron-binding capacity and UIBC ( < 0.0001) in deferasirox-treated patients compared to deferoxamine-treated patients. Immunological changes between two treated groups showed insignificant differences in levels of complements (C3 and C4) and immunoglobulin levels (IgM, IgG, and IgA) > 0.05.
This study indicated that deferasirox is more effective than deferoxamine regarding the iron overload but not in the immunological profile in patients with blood transfusion-dependent β-thalassemia.
β地中海贫血是一组遗传性(常染色体隐性)血液系统疾病,在地中海地区较为普遍,是由于血红蛋白β链合成缺陷所致。本研究的目的是比较地拉罗司和去铁胺对依赖输血的重型和中间型β地中海贫血患者铁过载及免疫变化的影响。
本研究纳入64例已知重型或中间型β地中海贫血患者,这些患者均接受过输血及铁螯合剂治疗。对接受去铁胺和地拉罗司治疗的患者评估血清铁蛋白、血清铁、血清总铁结合力、不饱和铁结合力(UIBC)及免疫参数。
与接受地拉罗司治疗的患者(3000.62±188.23 ng/dL;P<0.0001)相比,接受去铁胺治疗的患者血清铁蛋白水平较高(8160.33±233.75 ng/dL),而且接受地拉罗司治疗的患者与接受去铁胺治疗的患者相比,血清铁、总铁结合力及UIBC也存在显著差异(P<0.0001)。两个治疗组之间的免疫变化显示补体水平(C3和C4)及免疫球蛋白水平(IgM、IgG和IgA)无显著差异(P>0.05)。
本研究表明,在地拉罗司治疗依赖输血的β地中海贫血患者时,在铁过载方面比去铁胺更有效,但在免疫方面并非如此。