Treatment

Therapeutic phlebotomy and chelation therapy are the primary options for iron removal.

• Address the underlying cause (e.g., alcohol use disorder) to stop iron loading.

• Consider iron removal on a case-by-case basis.

• First-line treatment for hereditary hemochromatosis (including asymptomatic patients)

• Consider in patients with secondary iron overload (e.g., for patients with symptomatic porphyria cutanea tarda)

• Inability to tolerate the procedure (e.g., due to anxiety)

• Low hemoglobin (e.g., due to iron-loading anemia)

• Conditions sensitive to fluid shifts (e.g., end-stage liver disease, congestive heart failure)

• Initial phase: blood removed weekly

• Maintenance phase: blood removed 3–4 times per year

• First-line treatment for secondary iron overload due to iron-loading anemia

• Consider for hereditary hemochromatosis refractory to therapeutic phlebotomy and patients with contraindications to phlebotomy.

deferoxamine, deferasirox, or deferiprone

Significant risk of adverse effects

E.g., renal toxicity, hepatic toxicity, retinopathy, ototoxicity, neutropenia, agranulocytosis, gastrointestinal bleed, rash

Check renal function prior to administration of chelating agents because of the risk of nephrotoxicity and renal accumulation.

Drugs that delete iron (Fe) in hemochromatosis: deFeroxamine, deFerasirox, deFeriprone

• Decompensated cirrhosis

• Hepatocellular carcinoma

• Advise avoidance of iron and vitamin C supplements —> Vitamin C increases intestinal iron absorption.

• Encourage strict avoidance of alcohol.

• No need to reduce dietary iron

• Early treatment of iron overload may:

  • Improve fatigue and skin hyperpigmentation

  • Reverse early organ damage (e.g., elevated liver chemistries, early cardiomyopathy)

  • Increase life expectancy