Hemochromatosis

Abstract

Diseases of iron storage encompass a spctrum of disorders, the classic example of which is idiopathic hemochromatosis. In this entity, massive iron accumulation is associated pathologically with widespread hemosiderin deposition and fibrosis, and clinically with liver cirrhosis, skin pigmentation, diabetes and cardiac failure. Most people feel that this form of iron overload is genetic in origin. However, lesser degrees of iron overload and organ dysfunction, commonly termed 'hemosiderosis', may have a variety of etiologies and are considered 'acquired'. Differentiation clinically along this spectrum may be difficult, but is important in treatment. This paper deals with the following aspects: iron metabolism, etiology, clinical features, diagnosis, treatment, and prognosis. (41 references).