![]() Survival benefits of iron depletion, if any, have been difficult to demonstrate because a small minority of persons with hemochromatosis have or will ever develop cirrhosis. This index is rarely useful in the current era of genetic testing. ![]() Liver iron concentration was most useful when the iron concentration/age (hepatic iron index) was used as a diagnostic test for hemochromatosis. It should be considered in C282Y homozygotes with SF 1000 μg/L or greater and in patients with noniron risk factors for liver disease (eg, alcohol, obesity, viral hepatitis). 25 Liver biopsy has moved from a diagnostic test to a prognostic test in many patients with hemochromatosis. 23, 24 Hepatic elastography is a noninvasive tool that may be useful to monitor this potential benefit without the need for liver biopsy. 21 Several studies in which the authors evaluated liver biopsies before and after phlebotomy therapy demonstrated that hepatic fibrosis can be reversed by phlebotomy therapy. 21, 22 The presence of symptoms related to iron overload in C282Y homozygotes found in a population screening study was 28.4% in men and 1.2% in women. In population-based studies, these complications occur in approximately 5% of male and less than 1% of female C282Y homozygotes. Liver fibrosis, cirrhosis, and hepatocellular carcinoma are the most serious complications of iron overload. Toxicity often dose dependent gastrointestinal symptoms transaminase elevations elevation of serum creatinine rash rare hearing, vision abnormalities severe (sometimes fatal) liver, kidney, or marrow toxicity Stool as chelate daily iron excretion variableįew reports of use in hemochromatosis to achieve iron depletion no clear benefit for patients with iron-induced cardiomyopathy expensive Good chelation of hepatic iron consider its use in patients with inadequate venous access or intolerant of phlebotomy Infusion site reactions hearing, vision, growth, skeletal abnormalities zinc deficiency Yersinia infection ![]() Urine as chelate daily iron excretion variableįew reports of use in hemochromatosis, mostly to achieve iron depletion inadequate chelation of cardiac iron in some cases expensive Much clinical experience in iron overload patients without hemochromatosis widely available consider its use in patients intolerant of phlebotomy Transient hypovolemia fatigue increases iron absorption citrate reaction iron deficiency if monitoring inadequate or inappropriate Limited clinical experience requires special apparatus and facility, limited availability expensive Rapid, safe may be preferred for patients with severe iron overload Transient hypovolemia fatigue increases iron absorption iron deficiency if monitoring inadequate or inappropriate Requires repeated visits to health-care facility requires normal erythropoiesis some patients report intolerance Much experience effective on the part of the clinician, widely available, safe, inexpensive reversal of cirrhosis in some cases may improve left ventricular diastolic functionīlood as hemoglobin (1 mL of erythrocytes = 1 mg of Fe)Įxcellent for iron depletion good for maintenance The presence of symptoms or increased serum levels of liver enzymes are not a prerequisite for initiating phlebotomy therapy. New information from population studies has demonstrated that SF levels do not increase progressively in all patients, and thus observation with follow-up SF measurements can be considered as an alternative management strategy. The conventional approach is to treat all C282Y homozygotes with SF above the reference range. ![]() 18 The decision to treat C282Y homozygotes with moderate elevations of SF (200-1000 μg/L) should consider patient preferences and clinical judgment ( Table 1). It is widely agreed that persons with hemochromatosis whose SF is 1000 μg/L or greater should undergo phlebotomy to achieve iron depletion. 14 These observations from population screening studies may not be relevant to a C282Y homozygote with symptoms referred to initiate phlebotomy therapy. 13 An adult C282Y homozygote with a normal SF level at diagnosis is very unlikely to develop iron overload later. 16, 17 The degree of elevation of the SF level at presentation can be used to predict the risk of progression. 10-15 These studies demonstrate that not all C282Y homozygotes, including those with an increased SF level, are destined to have progressive iron overload. The authors of several large population-based studies have performed hemochromatosis genetic testing on participants many years into the study and used stored blood samples to measure SF over time in participants found to have C282Y homozygosity. ![]()
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