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Overview
Treatment of patients with hemochromatosis is initiated whenever an elevated transferrin saturation and serum ferritin (>200 mcg/l) has been demonstrated. The treatment consists of removal of iron by phlebotomy (Figure 14). One unit of blood (500 ML) typically contains 200-250 mg of iron. At phlebotomy, 500 mL of blood is removed weekly until serum iron and serum ferritin fall into the deficient range, and percent saturation of transferrin falls below 15%. It may take years to deplete the iron stores of individuals with symptoms, but with early diagnosis 30 or fewer phlebotomies are likely sufficient. Patients with cardiac disease may only tolerate removal of 0.5 units of blood weekly. Thereafter, the frequency of phlebotomy is reduced to maintain a serum ferritin of 50 mcg/l. Typically for maintenance, men will require phlebotomy 3-4 times a year and women 1-2 times per year. In all cases, hemoglobin needs to be monitored so that marked anemia is avoided. Phlebotomy should be continued for the life of the individual.
In rare cases, patients are unable to tolerate phlebotomy. In these instances chelation therapy with deferoxamine should be considered.
Patients should avoid iron supplementation and restrict their vitamin C and ethanol intake as these both facilitate iron absorption. In addition they should avoid raw shellfish, as they are more susceptible to Vibrio vulnificus infection. Therapy has many benefits. If initiated early, it will prevent cirrhosis and other complications of iron overload, as well as decreasing the risk of hepatocellular carcinoma . In addition to increasing life span, therapy should improve or alleviate almost all symptoms (except for hypogonadism and arthropathy).
Make an appointment today - call (410) 955-4166.
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Overview
Phlebotomy has been found to markedly improve symptoms of weakness, lethargy, and abdominal pain and to decrease hepatomegaly and serum aminotransferases. However, endocrine and arthropathic changes only improve in approximately 25% of patients. Glucose intolerance and insulin requirements decrease in some patients. No reversal of cirrhosis has been documented. There is no evidence that iron depletion by phlebotomy decreases the high incidence of hepatocellular carcinoma. Phlebotomy, however, increases survival in patients with pre-cirrhosis hemochromatosis who can be depleted of iron within 18 months of phlebotomy. Pre-cirrhotic patients depleted of iron with venesection have a normal life expectancy. Cancer surveillance should include yearly physical examination and biannual imaging with serum alpha-fetoprotein (Figure 15).
Liver transplantation is an appropriate therapy in patients with advanced cirrhosis due to hemochromatosis (Figure 16). However, survival is decreased when compared with patients transplanted for cirrhosis of other etiologies. One study, examining 22 patients with hemochromatosis, showed a median survival of 2.8 years after liver transplantation with the longest survival being 5.5 years. Percent transferrin saturation and serum ferritin fell within 6 months in all patients, and liver iron remained normal in the transplanted livers. However, the time period of follow-up was too short to determine the extent of iron re-accumulation.
Make an appointment today - call (410) 955-4166.
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