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Investigation and Management of Iron Overload

Source: www.healthservices.gov.bc.ca
Topic: Hemochromatosis
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Sort Desciption: Iron Overload Investigation and Management ... Scope This guideline provides recommendations for the investigation of iron overload and management of hemochromatosis. It applies to patients of all ages.

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Iron Overload Investigation and Management Effective Date: December 15, 2006 Scope This guideline provides recommendations for the investigation of iron overload and management of hemochromatosis. It applies to patients of all ages. Iron overload refers to all conditions where excessive amounts of iron accumulate in tissues resulting in parenchymal damage and organ dysfunction. The various forms of iron overload may be classified as: a) INHERITED HFE-related (occurs predominantly in people of European descent) Non-HFE-related (can occur in other ethnic groups but is uncommon) b) ACQUIRED Iron loading anemias, transfusion iron overload, etc. Note: The terminology varies in the literature. More recent references reserve the terms hereditary hemochromatosis and genetic hemochromatosis for HFE-related iron overload. The symbol HFE has been used for many years to designate the gene for hemochromatosis. R ecommendation 1 a) Patients who have symptoms or signs that might be caused by iron overload. These include patients with (unexplained): arthritis (including premature osteoarthritis) congestive heart failure or cardiomyopathy adult-onset diabetes persistent elevation of liver enzymes or cirrhosis secondary hypogonadism increased skin pigmentation b) Patients with persistently elevated serum ferritin not explained by an underlying inflammatory/ systemic disease. Notes: Serum ferritin typically has a wide reference range and may vary with age and gender: consult your laboratory. First degree relatives of a known case of HFE-related hemochromatosis should be offered DNA testing as the first step. Refer to Recommendation 3. Other groups at risk of iron overload include patients receiving long-term red cell transfusion support for chronic anemia and patients with porphyria cutanea tarda. Management of these patients should be discussed with a specialist. Serum ferritin levels may be elevated out of proportion to total body iron ...

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