Transferrin-immune complex disease.
A 71-year-old woman showed a highly unusual pattern of iron distribution in the organism which was associated with iron overload. The hallmark of this disease was an extreme hypersiderinemia, the serum iron reaching about 800 mug/100 ml. There was a pigment cirrhosis of the liver, bronzed skin containing hemosiderin, and diabetes mellitus. Paradoxically, hemosiderin was not detectable in bone marrow macrophages, sideroblasts and erythrocytes were reduced, and there was a decrease in radioiron utilization of erythropoiesis, thus indicating insufficient iron supply. The pathogenesis of this disorder based on the formation of an autoantibody with specificity for transferrin thus producing a circulating immune complex which bound the majority of serum iron. Immunosuppression achieved a partial remission including a recovery of the patient's general state, a rise in free transferrin, a decrease in serum iron, disappearance of hemosiderin in the liver, and a rise in erythrocyte production.
D000368:Aged; D001327:Autoimmune Diseases; D001379:Azathioprine / Q000627:therapeutic use; D001853:Bone Marrow / Q000378:metabolism; D004906:Erythrocyte Count; D005260:Female; D006801:Humans; D007105:Immune Complex Diseases / Q000188:drug therapy; D007501:Iron / Q000097:blood / Q000378:metabolism*; D011239:Prednisolone / Q000627:therapeutic use; D014168:Transferrin
D014168:Transferrin; D011239:Prednisolone; D007501:Iron; D001379:Azathioprine