Internal Distribution of Excess Iron and Sources of Serum Ferritin in Patients with Thalassaemia

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Abstract
Liver and spleen Fe concentrations, serum ferritin level and binding of S-ferritin to concanavalin A (Con A) were measured in 12 patients with thalassemia major or intermedia at the time of splenectomy. All these subjects had increased liver Fe concentration, most of them had hepatic fibrosis but none of them had histological evidence of chronic hepatitis. No patient had ascorbic acid deficiency. Serum ferritin concentration was increased in all cases, ranging from 266-5504 .mu.g/l. In all but 2 subjects most of the protein did not bind to Con A, thus behaving as tissue ferritin. There were highly significant correlations between serum ferritin concentration, amount of blood transfused and liver Fe concentration. On the average, Fe concentration in the liver was .apprx. 3 times that in the spleen. In patients with thalassemia major or intermedia most of the Fe is deposited in parenchymal tissues, and most of the S-ferritin derives by leakage from the cytosol of Fe-loaded parenchymal cells. S-ferritin is a valid index of liver Fe overload in thalassemic patients without complications such as viral hepatitis and/or ascorbic acid deficiency.