Zimelman A P, Miller A
Arch Intern Med. 1980 Jul;140(7):983-4.
A patient with both hereditary spherocytosis and hemochromatosis is described. At the time of the initial diagnosis of hereditary spherocytosis and shortly after splenectomy, 8 g of iron was removed by phlebotomy. During the next 15 years, the patient continued to accumulate excess iron despite splenectomy-induced remission of the hemolytic disorder. Thus the hemochromatosis in this patient was not secondary to the hereditary spherocytosis but rather represented a primary, ie, genetic abnormality of iron absorption.
本文描述了一位同时患有遗传性球形红细胞增多症和血色素沉着症的患者。在首次诊断出遗传性球形红细胞增多症时以及脾切除术后不久，通过放血去除了8克铁。在接下来的15年里，尽管脾切除术后溶血性疾病得到缓解，但患者仍继续蓄积过量的铁。因此，该患者的血色素沉着症并非继发于遗传性球形红细胞增多症，而是代表了一种原发性的，即铁吸收的基因异常。
