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It may lead to a decline in cardiac function
0%
0/27
It may improve with serial phlebotomy
7%
2/27
It may improve with calcium chelators
85%
23/27
It is associated with an increased risk for hepatocellular carcinoma
4%
1/27
It results in skin bronzing
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This patient's clinical presentation is consistent with hemochromatosis. Iron chelators such as deferoxamine, not calcium chelators, are used as treatment for this condition. Hemochromatosis is a disorder of iron absorption caused by mutations in the HFE gene. It commonly presents with abnormal liver enzymes, lethargy, and skin hyperpigmentation. Classical clinical manifestations of hemochromatosis are caused by iron deposition in the liver, heart, pancreas, and pituitary. Liver deposition leads to cirrhosis and an increased risk of hepatocellular carcinoma. Cardiac deposition leads to a restrictive cardiomyopathy, and pituitary deposition can cause amenorrhea and dysregulation of the hypothalamic-pituitary system. Iron deposition in the pancreas can lead to diabetes mellitus and malabsorption. Treatment includes repeated phlebotomy and administration of iron-chelating agents, such as deferoxamine. Crownover and Covey review hemochromatosis. They note that disease is significantly more common in men than women, with a ratio of 24:1. Upon diagnosis, liver biopsy should be performed to evaluate for the degree of cirrhosis. Patients with cirrhosis should be monitored frequently for hepatocellular carcinoma. Sanchez et al. discuss the risks of receiving blood donations from patients with hemochromatosis. Since the treatment for hemochromatosis involves repeated phlebotomy, patients with this condition have the opportunity to contribute frequently to blood banks. They conclude that patients with hemochromatosis are not more likely to transmit viral diseases via blood transfusion than other patients. Illustration A shows common effects of hemochromatosis on major organ systems. Illustration B is a photo of the hand of a patient with hemochromatosis (right) and a normal control (left). Skin bronzing is most notable on sun-exposed areas, such as the hands and face. Incorrect Answers: Answer 1: Untreated hemochromatosis can result in iron deposits in cardiac tissue, leading to congestive heart failure. Answer 2: Repeated phlebotomy is a cornerstone of treatment for hemochromatosis. Answer 4: Hemochromatosis is associated with an increased risk of hepatocellular carcinoma in patients that progress to cirrhosis. Answer 5: Hemochromatosis is often called "bronze diabetes," as iron deposition in the skin leads to bronzing.
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