ABSTRACT
Hemochromatosis implies progressive iron overload leading to fibrosis and organ failure. Diseases associated with ineffective erythropoeisis and increased iron absorption lead to predominantly parenchymal iron loading, whereas when the iron load has been acquired primarily parenterally, that is, by transfusion, there is predominantly reticuloendothelial (RE) cell involvement at least in the initial stages of loading. The iron overload seen in South African blacks was attributable to large amounts of "dietary" iron consumed with ethanol resulting from the brewing of "Kaffir beer" in large iron pots. Iron absorption is regulated via poorly understood mechanisms to maintain body iron stores at optimal levels. The inappropriate iron absorption may result from a genetically determined error of iron metabolism in which the basic metabolic defect leading to the increased iron absorption is unknown or from ineffective erythropoiesis. Cellular dysfunction and tissue injury appear to be related more closely to parenchymal than to RE iron loading.
