Literature DB >> 10942934

Juvenile hemochromatosis associated with B-thalassemia treated by phlebotomy and recombinant human erythropoietin.

M De Gobbi1, P Pasquero, F Brunello, P Paccotti, U Mazza, C Camaschella.   

Abstract

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.

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Year:  2000        PMID: 10942934

Source DB:  PubMed          Journal:  Haematologica        ISSN: 0390-6078            Impact factor:   9.941


  2 in total

Review 1.  Physiology and pathophysiology of iron in hemoglobin-associated diseases.

Authors:  Thomas D Coates
Journal:  Free Radic Biol Med       Date:  2014-04-12       Impact factor: 7.376

2.  A late presentation of a fatal disease: juvenile hemochromatosis.

Authors:  Cynthia Cherfane; Pauline Lee; Leana Guerin; Kyle Brown
Journal:  Case Rep Med       Date:  2013-09-11
  2 in total

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