Combined chelation treatment in patients with myelodysplastic syndrome and hereditary hemochromatosis – a case study

Česká verzia

Authors: J. Vondráková ¹; J. Procházková ¹; K. Indrák ¹; L. Kučerová ²
Authors place of work: Hemato-onkologická klinika Fakultní nemocnice a Lékařská fakulta Univerzity Palackého, Olomouc Přednosta: prof. MUDr. Karel Indrák, DrSc. ; Ústav patologie FN a LF UP, Olomouc Přednosta: doc. MUDr. Martin Tichý, CSc. ²
Published in the journal: Prakt. Lék. 2008; 88(3): 166-169
Category: Case Report

Summary

There is currently great interest in the diagnosis and treatment of iron overload syndromes. These syndromes are classified as primary (hereditary), for example hereditary hemochromatosis, or secondary (acquired), for example transfusional iron overload. An imbalance between iron uptake and iron elimination leads to its accumulation in the body and the toxic effect of iron overload causes tissue damage (heart, liver, endocrine organs) and can lead to end-organ failure. Therefore, research has focused on means of preventing iron-related morbidity and mortality. Therapeutic management of hereditary hemochromatosis involves venesection, while for transfusion iron overload, iron chelation therapy is useful (deferoxamine, deferiprone or deferasirox) mainly given as monotherapy. We report a complicated case of a patient with hereditary hemochromatosis and simultaneous myelodysplastic syndrome, when venesection was not indicated. In this situation it was necessary to solve anemia and systemic iron overload (hepatopathy, cardiomyopathy, diabetes mellitus) at the same time. This case report demonstrates that combined chelation therapy with deferoxamine and deferiprone was successful, improved patient quality of life and was performed completely in the outpatient clinic.

Key words:
myelodysplastic syndrome, hemochromatosis, iron overload, combined chelation therapy.

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