What is the treatment for a patient with hyperferritinemia (elevated iron levels)?

From the Guidelines

Therapeutic phlebotomy is the primary treatment for patients with high iron levels, aiming to reduce ferritin levels to 50-100 lg/L, with weekly removal of 500ml of blood until normalization, followed by maintenance phlebotomies every 2-4 months. The decision to treat is straightforward for patients with evidence of liver disease or other end-organ manifestations, but for those without symptoms, treatment is often initiated due to its ease, safety, and potential societal benefits, such as blood donation 1.

Key Considerations

• Phlebotomy should be carried out by removing 400–500 ml of blood (200–250 mg iron) weekly or every two weeks, with adequate hydration before and after treatment, and avoidance of vigorous physical activity for 24 h after phlebotomy 1.
• Before the initiation of phlebotomy, patients should be assessed for complications including diabetes mellitus, joint disease, endocrine deficiency (hypothyroidism), cardiac disease, porphyria cutanea tarda, and osteoporosis 1.
• Vitamin C supplements and iron supplements should be avoided during treatment, as pharmacologic doses of vitamin C can accelerate mobilization of iron, potentially increasing pro-oxidant and free-radical activity 1.
• Regular monitoring of ferritin levels, liver function, and cardiac status is necessary throughout treatment to prevent complications like cirrhosis, diabetes, and cardiomyopathy 1.

Alternative Treatments

• Iron chelation therapy is an alternative for those who cannot tolerate phlebotomy, with medications like deferasirox (Exjade, 20-40mg/kg/day), deferoxamine (Desferal, 20-60mg/kg/day subcutaneously), or deferiprone (Ferriprox, 75-100mg/kg/day) 1.
• Dietary modifications, including limiting red meat, vitamin C with meals, and alcohol consumption, may also be beneficial, although their impact is limited compared to phlebotomy or chelation therapy 1.

Underlying Cause Consideration

• For secondary hyperferritinemia caused by inflammation or liver disease, treating the underlying condition is essential rather than removing iron, as the primary goal is to address the root cause of the elevated ferritin levels 1.

From the FDA Drug Label

For patients with transfusional iron overload, measure serum ferritin monthly to assess for possible overchelation of iron. Use the minimum effective dose to achieve and maintain a low iron burden If the serum ferritin falls below 1000 mcg/L at 2 consecutive visits, consider dose reduction, especially if the deferasirox tablets dose is greater than 17.5 mg/kg/day If the serum ferritin falls below 500 mcg/L, interrupt therapy with deferasirox tablets and continue monthly monitoring.

The treatment for a patient with hyperferritinemia (elevated iron levels) is to use deferasirox tablets to reduce iron levels.

• Monitor serum ferritin levels monthly to assess for possible overchelation of iron.
• Use the minimum effective dose to achieve and maintain a low iron burden.
• Consider dose reduction if serum ferritin falls below 1000 mcg/L at 2 consecutive visits.
• Interrupt therapy if serum ferritin falls below 500 mcg/L and continue monthly monitoring 2.

From the Research

Treatment Options for Hyperferritinemia

• Therapeutic phlebotomy is used to remove excess iron and maintain low normal body iron stores, and it should be initiated in men with serum ferritin levels of 300 microg/L or more and in women with serum ferritin levels of 200 microg/L or more, regardless of the presence or absence of symptoms 3.
• Dietary management of hemochromatosis includes avoidance of medicinal iron, mineral supplements, excess vitamin C, and uncooked seafoods, which can reduce the rate of iron reaccumulation and help reduce complications of liver disease, diabetes mellitus, and Vibrio infection 3.

Iron Chelation Therapy

• Deferasirox is an effective oral chelator for managing iron overload in people with thalassaemia, and its efficacy is similar to that of deferoxamine, depending on the dose and ratio of deferasirox compared to deferoxamine 4.
• Hydroxyurea has been shown to have iron chelating effects and can be used in combination with other iron chelators like deferiprone and deferasirox to reduce iron from experimentally iron overloaded mice 5.
• Combined subcutaneous (deferoxamine) and oral (deferiprone) treatment seems to hold particular promise for iron mobilization using chelation and phlebotomy 6.

Patient Compliance with Phlebotomy Therapy

• Most patients with hemochromatosis diagnosed in medical care achieve iron depletion with phlebotomy, and one-third tolerate and adhere to weekly phlebotomy 7.
• Average compliance with maintenance therapy decreases 6.8% annually, and the mean follow-up after achieving iron depletion is 4.1 +/- 2.8 yr (range 0.6-9.7 yr) 7.

Key Considerations

• Patient compliance with phlebotomy therapy is crucial for effective management of iron overload associated with hemochromatosis 7.
• Iron chelation therapy should be individualized based on the patient's specific needs and circumstances, and deferasirox could be offered as the first-line option to individuals who show a strong preference for it 4.