Despite advances in the molecular understanding of hemochromatosis and the impact of C282Y on diagnosis, treatment remains simple, inexpensive, and safe.

The goal of therapy in patients with iron overload disorders is to remove the iron before it can produce irreversible parenchymal damage. [8] This is achieved via chelation therapy or venesection, depending on the underlying cause. [80] Because a normal life span can be expected if iron reduction is initiated before the development of cirrhosis, clinical suspicion and early diagnosis are essential.

The tetrad of cirrhosis, diabetes mellitus, hyperpigmentation of the skin, and cardiac failure may be evident in only a minority of patients. [81] Any patient admitted to the hospital with an isolated case of asthenia or with arthralgia or hypertransaminasemia should be examined by means of transferrin-saturation testing.

Cardiac manifestations of hereditary hemochromatosis could have sudden onset and could be poorly responsive to therapy. The hemochromatic etiology of the cardiomyopathy should be identified to ensure appropriate treatment.

A Cochrane database review of interventions for hereditary hemochromatosis found that phlebotomy remained the treatment of choice in those with hereditary hemochromatosis who required blood letting, but no data from randomized trials provided evidence of benefit from any form of blood letting in these patients. There was also insufficient evidence as to whether erythocytapheresis is beneficial or harmful compared with phlebotomy. [82] The investigators noted an overall low quality of evidence and a high risk of bias in the trials assessed, and none of the trials evaluated iron-chelating agents or provided mortality data beyond 1 year or long-term follow-up. [82]

Admission to the intensive care unit (ICU) may be warranted for patients who develop hepatic, cardiac, and infectious complications. Indications for inpatient care, preferably in an ICU, include the following:

Gastroesophageal bleeding

Hepatic encephalopathy

Sepsis

Congestive heart failure

Arrhythmias

Transfer considerations

In case of end-stage liver disease that is refractory to all methods of medical treatment, transferring the patient to a facility experienced in liver transplantation is preferable.

When the diagnosis of hepatocellular carcinoma is being considered or if the diagnosis is confirmed, transfer the patient to a cancer institution.