Martín María, Estornell Jordi, Orero Maite, Pérez José L, Ridocci Francisco, Martínez Vicente
Servicio de Cardiología, Hospital Central de Asturias, Asturias, Spain.
Rev Esp Cardiol. 2006 Jan;59(1):75-7.
Heart failure secondary to iron overload is the main cause of death in patients with beta-thalassemia major. Combination therapy with deferoxamine and deferiprone has been shown to be more effective than either drug used alone in patients with beta-thalassemia major and symptomatic cardiomyopathy. Although monitoring the response to chelation therapy is usually carried out by indirect measurement of the serum ferritin level or by direct determination of tissue iron content in biopsy specimens, magnetic resonance imaging (MRI) seems to be useful for noninvasive qualitative and quantitative assessment of iron deposition. We present a case in which the efficacy of double chelation therapy in a patient with beta-thalassemia major and heart failure was demonstrated by MRI.
铁过载继发的心力衰竭是重型β地中海贫血患者的主要死因。去铁胺和去铁酮联合治疗已被证明在重型β地中海贫血和有症状性心肌病患者中比单独使用任何一种药物更有效。尽管螯合治疗反应的监测通常通过间接测量血清铁蛋白水平或直接测定活检标本中的组织铁含量来进行,但磁共振成像(MRI)似乎有助于对铁沉积进行无创定性和定量评估。我们报告一例通过MRI证实双螯合疗法对重型β地中海贫血合并心力衰竭患者疗效的病例。