López D E, Kohan M, Ferreño D, Raffa M P, Prytyka A
Servicio de Clínica Médica, Hospital General de Agudos, Dr. T. Alvear, MCBA.
Acta Gastroenterol Latinoam. 1997;27(4):267-70.
Hereditary spherocytosis is a chronic hemolytic anemia that very infrequently produces severe iron overload. Only 15 cases of hereditary spherocytosis associated with hemochromatosis have been described previously. It was initially thought that hemochromatosis was the result of the increase of iron stores secondary to chronic hemolysis. Afterwards, it became apparent that iron overload could appear in patients splenectomized. This fact suggested that spherocytosis and idiopathic hemochromatosis could be inherited independently. We describe the case of a 45-year-old man, with known hereditary spherocytosis, splenectomized at 5 years of age, who developed iron overload which affected his heart, liver and pancreas.
遗传性球形红细胞增多症是一种慢性溶血性贫血,极少会导致严重的铁过载。此前仅报道过15例与血色素沉着症相关的遗传性球形红细胞增多症病例。最初认为血色素沉着症是慢性溶血继发铁储存增加的结果。后来发现,脾切除患者也会出现铁过载。这一事实表明,球形红细胞增多症和特发性血色素沉着症可能是独立遗传的。我们报告了一例45岁男性病例,他已知患有遗传性球形红细胞增多症,5岁时接受了脾切除术,后来出现了影响心脏、肝脏和胰腺的铁过载。