What is the treatment for hyperferremia (high iron levels)?

Treatment of Hyperferremia (High Iron Levels)

The primary treatment for hyperferremia (high iron levels) is therapeutic phlebotomy, which should be initiated in patients with elevated ferritin levels to reduce iron stores to normal levels and prevent complications of iron overload. 1, 2

Diagnostic Approach Before Treatment

• Determine if hyperferremia is due to iron overload by measuring transferrin saturation 1
• Consider genetic testing for hereditary hemochromatosis (HFE) if transferrin saturation is elevated 1
• Assess for end-organ damage through liver function tests and consider liver biopsy if cirrhosis is suspected or if ferritin >1000 μg/L 2

Treatment Protocol for Primary Iron Overload

Initial Therapeutic Phlebotomy

• Begin weekly therapeutic phlebotomy (removal of 400-500 mL of blood) as tolerated by the patient 2, 1
• Monitor hemoglobin and hematocrit before each phlebotomy session 2, 1
• Postpone phlebotomy if anemia develops until resolved 2, 1
• Check serum ferritin every 10-12 phlebotomies initially, more frequently as levels approach normal range 2, 1
• Continue therapeutic phlebotomy until ferritin reaches target of less than 50 μg/L 2, 1

Maintenance Therapy

• After reaching target ferritin levels, continue maintenance phlebotomies every 3-6 months 2, 1
• Maintain serum ferritin between 50-100 μg/L 2
• Alternative approach: cease phlebotomy and monitor ferritin, restarting when ferritin reaches upper limit of normal 2

Treatment of Secondary Iron Overload

For Secondary Iron Overload with Anemia

• Iron chelation therapy is the treatment of choice when phlebotomy is not feasible due to anemia 2
• Options include:
  • Deferoxamine (Desferal): 20-40 mg/kg/day via subcutaneous infusion 2, 3
  • Deferasirox (Exjade): orally administered iron-chelating drug approved for secondary iron overload due to ineffective erythropoiesis 2
• Monitor iron reduction through serum ferritin or consider liver biopsy to assess progress of therapy 2

For Specific Secondary Iron Overload Conditions

• Porphyria cutanea tarda: Phlebotomy is clearly indicated and results in reduction of skin manifestations 2
• Non-alcoholic fatty liver disease (NAFLD): Phlebotomy may improve parameters of insulin resistance and reduce elevated ALT levels 2
• Chronic hepatitis C: Phlebotomy not recommended for mild secondary iron overload (HIC < 2500 μg/g dry weight) 2

Dietary and Lifestyle Considerations

• Avoid iron supplements and iron-fortified foods 2, 1
• Limit vitamin C supplements to 500 mg/day as excessive vitamin C can increase iron absorption 2, 1
• Avoid excess alcohol consumption, which can worsen liver damage 1, 4
• Avoid raw shellfish due to risk of Vibrio vulnificus infection, particularly in patients with cirrhosis 2, 5
• Maintain a broadly healthy diet rather than strict iron restriction 2, 1

Monitoring and Follow-up

• Monitor serum ferritin monthly during initial therapy and every 3-6 months during maintenance 2, 1
• Perform regular screening for hepatocellular carcinoma in patients with cirrhosis 2, 1
• Early treatment before development of cirrhosis and diabetes improves survival to normal population levels 2, 1

Pitfalls and Caveats

• Avoid overchelation by careful monitoring of ferritin levels 1
• Recognize that certain clinical features respond differently to iron removal:
  • Responsive: malaise, fatigue, skin pigmentation, insulin requirements for diabetics, abdominal pain 2
  • Less responsive: arthropathy, hypogonadism 2
  • No response: advanced cirrhosis 2
• Alternative treatments like erythrocytapheresis may be more efficient but are less widely available 6