Tissue Iron Stores in Sideroblastic Anaemia
Journal Articles
Overview
Research
Identity
Additional Document Info
View All
Overview
abstract
An increase in tissue iron stores (chiefly in liver and bone marrow) has been a constantly reported finding in appropriately investigated cases of sideroblastic anaemia, and in almost 50 per cent of these cases the degree of the hepatic siderosis and the extent of fibrosis have been considered sufficient to warrant the designation haemochromatosis. Seventy per cent of these patients with haemochromatosis had the familial type of sideroblastic anaemia. Bottomley's (1962) analysis of case reports of pyridoxine‐responsive anaemia showed that haemochromatosis, too, had been diagnosed in about half these patients.In the majority of patients with an acquired form of sideroblastic anaemia, the anaemia apparently preceded the development of the haemosiderosis, which could therefore have resulted from an increase in the absorption of iron associated with a chronic anaemia and erythroid hyperplasia and/or from prolonged iron therapy with or without blood transfusions. However, in a few patients with non‐familial sideroblastic anaemia (Albahary and Boiron, 1959; Crosby and Sheehy, 1960; André, Jacob, Malassenet and Caroli, 1961) and in some with the familial form (Caroli, Bernard, Bessis, Combrisson, Malassenet and Breton, 1957; Lukl, Weidermann and Barbořík, 1958; Byrd and Cooper, 1961), the hepatic siderosis appears to have preceded the anaemia or to have been present to a greater extent than could be accounted for by therapy.Bishop and Bethell (1959) suggested that the presence of excess erythroblast iron might inhibit haemoglobin synthesis. Harriss, MacGibbon and Mollin (1965) have shown that the administration of iron (either by mouth or injection) increases the severity of the anaemia in experimental pyridoxine deficiency.These observations pose the following questions: does the increase in tissue iron precede the development of anaemia (if so, what is the underlying abnormality in iron metabolism?) or is it a sequel; in which case is it the result of an increased absorption of dietary iron and/or excessive iron therapy with or without multiple blood transfusion?This paper presents the results of an assessment of bone‐marrow and liver iron in 37 patients with sideroblastic anaemia, and of the measurement of iron absorption in 11 patients.
