What is the guideline treatment goal for ferritin levels in hemochromatosis?

Guideline Treatment for Hemochromatosis: Ferritin Goals

The guideline treatment for hemochromatosis targets a serum ferritin level of 50 μg/L during the induction phase and 50-100 μg/L during the maintenance phase, as recommended by the European Association for the Study of the Liver (EASL) in their 2022 clinical practice guidelines. 1

Treatment Phases and Ferritin Targets

Induction Phase

• Target: Serum ferritin of 50 μg/L (but not lower to avoid iron deficiency) 1
• Method: Weekly or fortnightly phlebotomy (400-500 ml) until target is reached 1
• Monitoring:
  • Check ferritin monthly or after every 4th phlebotomy
  • When ferritin falls below 200 μg/L, check after every 1-2 sessions 1
  • Monitor hemoglobin at each phlebotomy session 1, 2

Maintenance Phase

• Target: Serum ferritin 50-100 μg/L 1, 2
• Method: Phlebotomy every 1-4 months depending on individual iron reaccumulation rate 1
• Monitoring: Check ferritin every 6 months to adjust treatment schedule 1

Important Clinical Considerations

Phlebotomy Adjustments

• If hemoglobin <12 g/dl: Decrease phlebotomy frequency or reduce volume 1
• If hemoglobin <11 g/dl: Discontinue phlebotomy and reassess 1
• Avoid ferritin falling below 50 μg/L to prevent symptomatic iron deficiency 1, 2

Variations in Guidelines

While there is strong consensus around the 50 μg/L target for the induction phase, some variations exist:

• American Association for the Study of Liver Diseases: 50-100 μg/L for both phases 1
• British Society for Haematology: 20-30 μg/L for induction and <50 μg/L for maintenance 1
• Danish guidelines: <100 μg/L for induction and 50-100 μg/L for maintenance 1
• American College of Gastroenterology: 50-100 μg/L for all patients 1, 2

Special Populations

• Elderly patients: More relaxed targets may be appropriate (ferritin <200 μg/L for women and <300 μg/L for men) as lower targets are often poorly tolerated 1
• Patients with cirrhosis: Require regular screening for hepatocellular carcinoma 2

Monitoring Recommendations

• Hemoglobin: Check at each phlebotomy session 1, 2
• Ferritin: Monitor to avoid both iron overload and iron deficiency 1
• Transferrin saturation: While specific targets are lacking, monitoring is recommended 1

Potential Pitfalls

• Overtreatment: Reducing ferritin below 50 μg/L can increase dietary iron absorption and cause symptomatic iron deficiency 1, 2
• Undertreatment: Inadequate iron removal can lead to continued organ damage 1
• Poor compliance: Patient adherence to maintenance therapy decreases by approximately 6.8% annually 3

Adjunctive Measures

• Proton pump inhibitors (PPIs) may reduce iron absorption and decrease phlebotomy requirements during maintenance phase 1
• Avoid iron-fortified foods, limit red meat consumption, and avoid raw shellfish 2
• Limit vitamin C supplementation to <500 mg/day to avoid accelerating iron mobilization 2

By following these evidence-based guidelines for ferritin targets, hemochromatosis can be effectively managed to prevent complications of iron overload including hepatic cirrhosis, primary liver cancer, diabetes mellitus, and cardiomyopathy.