Treatment

Removal of excess iron by regular phlebotomy greatly reduces the mortality from cardiac and hepatic failure, although hepato-cellular carcinoma accounts for a substantial proportion of deaths in those with established disease. Early diagnosis is therefore a priority, as patients identified and treated before the onset of cirrhosis of the liver have a normal life expectancy.

Phlebotomy should be at a rate of 450 mL of blood each week, but the rate may need to be reduced if anaemia develops. Weekly phlebotomy will need to be continued for at least 6 months to remove a total iron excess, which is usually greater than 5 g in established symptomatic disease but may be more than 20 g. The amount of iron removed before anaemia develops should be calculated (see above). Since the advent of genetic testing, confirmation of iron overload by liver biopsy is not necessary in the absence of liver damage and quantitative phlebotomy provides the only practical way of confirming the presence of iron overload.

When iron stores are exhausted, the frequency of phlebotomy should be reduced to two to four units each year, to continue indefinitely. The aim is to maintain a normal transferrin saturation (< 50%) and a serum ferritin in the low normal range (< 50 |lg/L).

Fatigue and transaminase elevation usually reverse on venesection. In some patients, diabetes mellitus, hypogonadism and arthralgia improve, but cirrhosis and arthritis are not reversible.

Iron chelation with DFX given as a continuous intravenous infusion (see below) may have a limited role in the short-term management of patients with life-threatening cardiac failure.

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