Introduction 3 Measuring iron levels 1 Table 1 Comparison of methods for evaluating iron overload   Invasive Cost Limitation Validation Ferritin No Low Infection/inflammation Yes LIC Yes Median Fibrosis/cirrhosis Yes SQUID No High Not easily available Yes MRI No High Dedicated person Yes LIC  SQUID  MRI There are several methods of assessing body iron load. As 90% of excess iron is deposited in the liver, most techniques focus on measuring liver iron levels, and it is widely accepted that liver iron content (LIC) provides an accurate measure of whole-body iron concentration. The invasive nature of the liver biopsy means that other markers such as serum ferritin levels are frequently employed. Other approaches using biomagnetic susceptometry and magnetic resonance imaging (MRI) are also being assessed in order to identify an accurate, low-risk, and convenient approach to assessing patient iron status. Ferritin Serum ferritin is easily measured using a commercially available kit. A ferritin constantly below 2,500 mg/l has been shown to reduce the risk of cardiac complications, but a target value of 1,000 mg/l is recommended. Factors such as inflammation, ascorbate status, and hepatitis can affect serum ferritin levels. Therefore, results should be interpreted with caution and a trend in the evolution of serial measurements is a better index than day-to-day variation. In patients with transfusion-dependent diseases, chelation should be initiated after 10–20 blood transfusions or when ferritin level rises above 1,000 mg/l. Liver biopsy 1 8 Fig. 1  blue  Liver biopsy is an invasive technique that is associated with some pain and at risk of hemorrhage and infection. It is not indicated for routine assessment. Superconducting quantum interference device (SQUID) 6 Magnetic resonance imaging (MRI) 2 13 3 13 Fig. 2 13  top four images  bottom four images TE  Fig. 3 a dark tissue b 13  Iron chelation therapy 6 2 Table 2 4 Characteristics Deferoxamine (Desferal) Deferiprone (Ferriprox) Deferasirox (Exjade) Route of administration Subcutaneous or intravenous Oral Oral Half-life 20 min 2–3 h 8–16 h Routes of iron excretion Urine/stool Urine stool Dose range 20–60 mg/kg per day 50–60 mg/kg per day 20–30 mg/kg/day Guidelines for monitoring therapy Audiometry and eye exams annually Complete blood count weekly; ALT level monthly for first 3–6 months then every 6 months Serum creatinine level monthly; ALT level monthly Serum ferritin Serum ferritin Serum ferritin Assessment of liver iron annually Assessment of liver iron annually Assessment of liver iron annually Assessment of cardiac iron annually after 10 years of age Assessment of cardiac iron annually after 10 years of age Assessment of cardiac iron annually after 10 years of age Advantages Long-term experience Orally active Orally active Effective in maintaining normal or near-normal iron stores Safety profile well established Once-daily administration Reversal of cardiac disease with intensive therapy Enhanced removal of cardiac iron Demonstrated equivalency to deferoxamine at higher doses May be combined with deferiprone May be combined with deferoxamine Trials in several hematologic disorders Disadvantages Requires parenteral infusion May not achieve negative iron balance in all patients at 75 mg/kg per day Limited long-term data Ear, eye, bone toxicity Risk of agranulocytosis Need to monitor renal function Poor compliance  May not achieve negative iron balance in all patients at highest dose Deferoxamine mesylate (Desferal) is the current standard for iron chelation therapy. The long-term efficacy of this approach has been demonstrated, limiting organ damage and preventing premature death. Deferoxamine has a very short half-life in plasma (5–10 min). Therefore, deferoxamine is administered parenterally, either subcutaneously 8–12 h a day at least 5 days a week, or intravenously, through a Port-a-Cath for continuous infusion. Low compliance with the prolonged subcutaneous administration is the main reason of ineffective treatment. Toxic side effects such as auditory, ocular, and neurotoxic abnormalities are also drawbacks in the therapy. 7 9 1 9 10 11 2 3 12 Discussion 5