Assis Reijâne Alves de, Kassab Carolina, Seguro Fernanda Salles, Costa Fernando Ferreira, Silveira Paulo Augusto Achucarro, Wood John, Hamerschlak Nelson
Einstein (Sao Paulo). 2013 Dec;11(4):528-32. doi: 10.1590/s1679-45082013000400022.
To report a case of iron overload secondary to xerocytosis, a rare disease in a teenager, diagnosed, by T2* magnetic resonance imaging. We report the case of a symptomatic patient with xerocytosis, a ferritin level of 350ng/mL and a significant cardiac iron overload. She was diagnosed by T2* magnetic resonance imaging and received chelation therapy Ektacytometric analysis confirmed the diagnosis of hereditary xerocytosis. Subsequent T2* magnetic resonance imaging demonstrated complete resolution of the iron overload in various organs, as a new echocardiography revealed a complete resolution of previous cardiac alterations. The patient remains in chelation therapy. Xerocytosis is a rare autosomal dominant genetic disorder characterized by dehydrated stomatocytosis. The patient may present with intense fatigue and iron overload. We suggest the regular use of T2* magnetic resonance imaging for the diagnosis and control of the response to iron chelation in xerocytosis, and we believe it can be used also in other hemolytic anemia requiring transfusions.
报告一例继发于口形细胞增多症(一种青少年罕见疾病)的铁过载病例,该病例通过T2磁共振成像确诊。我们报告了一例有症状的口形细胞增多症患者,其铁蛋白水平为350ng/mL,存在显著的心脏铁过载。她通过T2磁共振成像被确诊,并接受了螯合疗法。血细胞比容分析证实了遗传性口形细胞增多症的诊断。随后的T2磁共振成像显示各个器官的铁过载完全消退,因为新的超声心动图显示之前的心脏改变已完全消退。该患者仍在接受螯合疗法。口形细胞增多症是一种罕见的常染色体显性遗传病,其特征为口形红细胞增多。患者可能出现严重疲劳和铁过载。我们建议定期使用T2磁共振成像来诊断口形细胞增多症并控制对铁螯合治疗的反应,并且我们认为它也可用于其他需要输血的溶血性贫血。