Iron overlo ad – recent advances in pathogenesis and tre atment

Česká verzia

Authors: J. Čermák ^1,2
Authors place of work: Ústav hematologi e a krevní transfuze Praha, zastupující ředitel prof. Ing. Jan E. Dyr, DrSc., 2Česká hematologická společnost České lékařské společnosti J. E. Purkyně, předseda doc. MUDr. Jaroslav Čermák, CSc. ¹
Published in the journal: Vnitř Lék 2009; 55(Suppl 1)(Supplementum 1): 59-63

Summary

Iron overlo ad may result as a consequence of incre ased iron income or deffective iron utilizati on. The most common re ason in o ur regi on is hereditary hemochromatosis or red blo od cell transfusi ons dependent anemi as with a high rate of ineffective erythropo i esis (eg. myelodysplastic syndrome). A key moment for the development of the toxicity ca used by iron overlo ad is incre ased iron rele ase into circulati on. An exceeded transferrin saturati on le ads to incre ased amo unt of non‑transferrin bo und iron in circulati on and one of its components, so called labile plasmatic iron may initi ate lipid peroxidati on resulting in cellular destructi on. Basic laboratory investigati ons for the di agnosis of iron overlo ad are serum ferritin and transferrin saturati on. NMR of liver or myocardi um serves as a useful to ol for non‑invasive qu antificati on of tissue iron. The tre atment of hereditary hemochromatosis is based on combinati on of erythrocytopheresis and chelati on therapy. Administrati on of iron chelators represents the tre atment of cho ice in iron lo aded anemi as. Desferi oxamine, deferiprone and deferasirox are the three currently available iron chelators. The aim of chelati on therapy sho uld be not only removal of incre ased body iron stores but also a preventi on of development of iron overlo ad and toxic effect of „free“ iron.

Key words:
iron – iron overlo ad – hereditary hemochromatosis – transferrin saturati on – labile plasmatic iron – serum ferritin – chelati on therapy

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