Tsironi Maria, Assimakopoulos George, Polonofi Katerina, Rigaki Kalliopi, Aessopos Athanassios
Thalassaemia Unit, Department of Internal Medicine, Sparta General Hospital, Sparta, Greece.
Hemoglobin. 2008;32(1-2):29-34. doi: 10.1080/03630260701680474.
The benefits of combined deferoxamine (DFO) and deferiprone (L1) chelation therapy, focusing on reducing myocardial iron loading, have been widely reported. Herein, we present the efficacy of combined chelation and its effects on iron load indices. Five thalassemia major (TM) patients who were undergoing chelation monotherapy with DFO were enrolled. Inclusion criteria were magnetic resonance imaging (MRI) T2* values, indicating serious heart and/or liver transfusional hemosiderosis. Combined therapy was started with the same dose of DFO and the addition of L1. The MRI T2* studies were repeated 18 months later. An Echo-Doppler study was performed in order to further evaluate the left ventricular (LV) systolic function. Within the 18 months' follow-up period, there was a significant statical decrease in mean serum ferritin levels. All patients increased their MRI T2* liver values, while two patients with very low MRI T2* also increased their myocardial values. The MRI ejection fraction (EF) and Echo-Doppler study measurements confirmed the improvement of systolic function. No adverse effects were reported. Combined L1 and DFO therapy seems to be effective in reducing iron excess in organ iron overloaded thalassemic patients. Magnetic resonance imaging can accurately quantify iron load, while echocardiography remains a reliable monitoring technology.
联合去铁胺(DFO)和去铁酮(L1)螯合疗法在减少心肌铁负荷方面的益处已被广泛报道。在此,我们展示联合螯合疗法的疗效及其对铁负荷指标的影响。招募了5名正在接受DFO螯合单一疗法的重型地中海贫血(TM)患者。纳入标准为磁共振成像(MRI)的T2值,提示存在严重的心脏和/或肝脏输血性含铁血黄素沉着症。联合疗法开始时使用相同剂量的DFO并加用L1。18个月后重复进行MRI T2研究。进行了超声多普勒研究以进一步评估左心室(LV)收缩功能。在18个月的随访期内,平均血清铁蛋白水平有显著的统计学下降。所有患者的肝脏MRI T2值均升高,而两名MRI T2值极低的患者其心肌值也有所升高。MRI射血分数(EF)和超声多普勒研究测量结果证实了收缩功能的改善。未报告不良反应。联合L1和DFO疗法似乎对减少器官铁过载的地中海贫血患者体内的铁过量有效。磁共振成像能够准确量化铁负荷,而超声心动图仍然是一种可靠的监测技术。