Iron Deficiency Anemia Associated with an Error of Iron Metabolism in Two Siblings *

This report describes the clinical and hemato- logical characteristics of two young siblings with chronic hypochromic microcytic anemia and high serum iron concentrations. Further tests have revealed that neither patient suffers from thalas- semia, hemoglobinopathy, or pyridoxine-respon- sive anemia. Subsequent examinations have disclosed an error in iron metabolism in these pa- tients characterized by massive hepatic iron depo- sition and absent stainable bone marrow iron stores. The association of this discrepancy with the chronic hypochromic anemia present in these two siblings is unique and has stimulated a series of special studies concerning their iron metabo- lism.