Vnitr Lek 2009, 55(Suppl 1):59-63

Iron overload - recent advances in pathogenesis and treatment

J. Čermák^1,2

¹ Ústav hematologie a krevní transfuze Praha, zastupující ředitel prof. Ing. Jan E. Dyr, DrSc.

² Česká hematologická společnost České lékařské společnosti J. E. Purkyně, předseda doc. MUDr. Jaroslav Čermák, CSc.

Iron overload may result as a consequence of increased iron income or deffective iron utilization. The most common reason in our region is hereditary hemochromatosis or red blood cell transfusions dependent anemias with a high rate of ineffective erythropoiesis (eg. myelodysplastic syndrome). A key moment for the development of the toxicity caused by iron overload is increased iron release into circulation. An exceeded transferrin saturation leads to increased amount of non-transferrin bound iron in circulation and one of its components, so called labile plasmatic iron may initiate lipid peroxidation resulting in cellular destruction. Basic laboratory investigations for the diagnosis of iron overload are serum ferritin and transferrin saturation. NMR of liver or myocardium serves as a useful tool for non-invasive quantification of tissue iron. The treatment of hereditary hemochromatosis is based on combination of erythrocytopheresis and chelation therapy. Administration of iron chelators represents the treatment of choice in iron loaded anemias. Desferioxamine, deferiprone and deferasirox are the three currently available iron chelators. The aim of chelation therapy should be not only removal of increased body iron stores but also a prevention of development of iron overload and toxic effect of "free" iron.

Keywords: iron; iron overload; hereditary hemochromatosis; transferrin saturation; labile plasmatic iron; serum ferritin; chelation therapy

Received: May 4, 2009; Published: February 1, 2009  Show citation