Chelation therapy for iron overload

Abstract

Iron overload is characterized by excessive iron deposition and consequent injury and dysfunction of the heart, liver, anterior pituitary, pancreas, and joints. Because physiologic mechanisms to excrete iron are very limited, patients with iron overload and its complications need safe, effective therapy that is compatible with their coexisting medical conditions. The availability of three licensed iron chelation drugs (one parenteral, two oral) and the development and clinical investigation of other oral chelators represent new opportunities to prevent or manage iron overload in patients with heritable types of severe anemia, such as beta-thalassemia major and sickle cell disease, and for the formulation of alternatives to phlebotomy therapy for patients with iron overload associated with the HFE gene and other adult age-of-onset types of hemochromatosis, African iron overload, and African-American iron overload.

Correlation of liver enzymes with serum ferritin levels in beta-thalassemia major

“…8 Barton JC et al noted similar results as serum ferritin increases liver enzymes also increases. 9 Limitation of our study is that we did not included controls which could have better given us comparative results as compared to normal subjects.…”

Manipulation of the hepcidin pathway for therapeutic purposes

“…This is an effective and inexpensive treatment for many patients, but not all patients tolerate phlebotomy. 54 Iron-loading anemias such as β-thalassemia are treated by iron chelation but this treatment can have serious side effects. 55 As hepcidin deficiency causes iron overload in both of these disorders, agents that can mimic hepcidin function or potentiate its endogenous production would be expected to prevent systemic accumulation of iron.…”

How I treat hemochromatosis

“…Clinical trials must be designed to determine whether oral chelation therapy can eliminate hepatic iron deposits without causing additional liver injury and whether hepatic fibrosis and cirrhosis caused by iron overload can be reversed. 61 Treatment schedules for maintaining low body iron stores after iron depletion has been achieved must also be established. 61 …”