Non-transferrin plasma iron in beta-thalassaemia/Hb E and haemoglobin H diseases.

Non-transferrin plasma iron concentrations were determined in 45 normal controls and in 37 patients with Hb H disease and 104 patients with beta-thalassaemia/Hb E disease. This revealed that non-transferrin plasma iron exists in cases with severe iron overload, more striking in beta-thalassaemia/Hb E than in Hb H disease. Non-transferrin plasma iron is associated with higher transferrin iron saturation and higher plasma ferritin levels. The most striking finding was the significantly higher non-transferrin plasma iron in splenectomized patients with beta-thalassaemia/Hb E disease than in the non-splenectomized patients. In view of the potential toxicity of non-transferrin iron, this fraction of iron may be responsible for tissue damage in these patients especially after splenectomy.