Abstract
This study on serum ferritin levels ind urinary iron excretion after 12h subcutaneous infusion of desferrioxamine in 10 thalassemia intermedia patients shows that even nontransfusion-dependent patients may have positive iron balance resulting in iron overload from 5 years of age. However, the iron overload found in these patients appears to be much lower than in age matched patients with transfusion-dependent thalassemia major. Iron overload increases with advancing age, as shown by increasing serum ferritin levels and desferrioxamine-induced urinary iron elimination. After a six month trial of 12h continuous subcutaneous desferrioxamine administration there was a significant decline in serum ferritin levels.
From this study it seems that iron chelation is indicated in thalassemia intermedia patients over 5 years of age in order to prevent iron accumulation. However, the appropriate treatment schedule should be tailored to the individual needs of each patients, established by close monitoring of serum ferritin levels and desferrioxamine-induced urinary iron elimination.
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This work was supported in part by grants from Assessorato Igiene e Sanità Regione Autonoma della Sardegna, N.I.H. grant number 5 R01 HL-24173-02, C.N.R. subprogetto finalizzato “Malattie Ereditarie dell'Eritrocita” contract number 80-02252.83
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Cossu, P., Toccafondi, C., Vardeu, F. et al. Iron overload and desferrioxamine chelation therapy in β-thalassemia intermedia. Eur J Pediatr 137, 267–271 (1981). https://doi.org/10.1007/BF00443255
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DOI: https://doi.org/10.1007/BF00443255