Combined chelation treatment in patients with myelodysplastic syndrome and hereditary hemochromatosis – a case study
Authors:
J. Vondráková 1; J. Procházková 1; K. Indrák 1; L. Kučerová 2
Authors‘ workplace:
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:
Prakt. Lék. 2008; 88(3): 166-169
Category:
Case Report
Overview
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.
Sources
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Labels
General practitioner for children and adolescents General practitioner for adultsArticle was published in
General Practitioner
2008 Issue 3
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