Juvenile hemochromatosis associated with B-thalassemia treated by phlebotomy and recombinant human erythropoietin
De Gobbi, M.; Pasquero, P.; Brunello, F.; Paccotti, P.; Mazza, U.; Camaschella, C.
Haematologica 85(8): 865-867
2000
ISSN/ISBN: 0390-6078 PMID: 10942934 Document Number: 516624
Juvenile hemochromatosis is a rare genetic disorder that causes iron overload. Clinical complications, which include liver cirrhosis, heart failure, hypogonadotropic hypogonadism and diabetes, appear earlier and are more severe than in HFE-related hemochromatosis. This disorder, therefore, requires an aggressive therapeutic approach to achieve iron depletion. We report here the case of a young Italian female with juvenile hemochromatosis who was unable to tolerate frequent phlebotomy because of coexistent ss-thalassemia trait. The patient was successfully iron-depleted by combining phlebotomy with recombinant human erythropoietin.