DOES RAPIDLY PROGRESSIVE IRON OVERLOAD IN A YOUNG GIRL WITH SIDEROBLASTIC ANEMIA ALSO SIGNIFY THE PRESENCE OF HEREDITARY HEMOCHROMATOSIS

A severely anemic 3-year-old girl with refractory sideroblastic anemia and fulminant, fatal hemochromatosis is described. The patient had tr ansfusion-dependent anemia with clinical cardiac, liver, and endocrine dysfunction that resulted from iron loading. The patient was minimall y transfused, and deferoxamine chelation was started at age 34 months. Despite treatment, the patient died at age 46 months as a result Of s evere iron overload. Sideroblastic anemia and iron overload in childho od are reviewed, and a pathophysiologic mechanism for the patient's cl inical course is postulated.