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

Treatment of Hyperferremia (Elevated Iron Levels)

Phlebotomy is the primary treatment for elevated iron levels (hyperferremia), with a target ferritin level of 50-100 μg/L. 1

Treatment Options Based on Cause

Hereditary Hemochromatosis and Primary Iron Overload

• Weekly therapeutic phlebotomy is the first-line treatment for patients with hemochromatosis and iron overload 1
• Continue phlebotomy until reaching target ferritin levels of 50-100 μg/L 1
• After achieving target levels, implement maintenance phlebotomy to prevent reaccumulation of iron 1
• Monitoring should occur every 3 months, with more frequent measurements as ferritin approaches normal range 1

Secondary Iron Overload (Transfusion-Dependent)

• Iron chelation therapy is recommended for transfusion-dependent patients with ferritin levels >1,000 ng/mL 1
• Chelation should be initiated when patients require ≥2 units of blood per month for more than one year 1
• For patients with myelodysplastic syndromes (MDS), chelation is particularly beneficial for those with low-risk disease and life expectancy >1 year 1
• Iron chelation therapy should continue as long as transfusion therapy is needed 1

Specific Chelation Agents

Deferoxamine

• Indicated for treatment of transfusional iron overload in patients with chronic anemia 2
• Not indicated for primary hemochromatosis (phlebotomy is preferred) 2
• Typical dosing: 20-60 mg/kg/day via subcutaneous infusion over 8-12 hours, 5-7 times weekly 2
• Maximum daily dose: 60 mg/kg/day for adults and 40 mg/kg/day for pediatric patients 2

Deferasirox

• Oral iron chelator approved for transfusional iron overload 3
• Dosing based on liver iron concentration (LIC), typically 5-30 mg/kg/day 3
• Has shown effectiveness in reducing serum ferritin and LIC in patients with MDS and other anemias 3

Monitoring Treatment

• Monitor serum ferritin and transferrin saturation regularly 1
• For patients on chelation therapy, monitor every 3 months at minimum, monthly if possible 1
• Liver imaging (MRI) and SQUID are useful but not essential monitoring techniques 1
• Monitor organ function periodically in patients with significant iron overload 1

Additional Considerations

• Avoid vitamin C supplements during treatment for iron overload as they may increase iron toxicity 1
• Avoid iron supplements and iron-fortified foods 1
• No specific dietary restrictions are necessary beyond avoiding iron supplements 1
• Raw shellfish should be avoided in patients with hemochromatosis due to risk of Vibrio vulnificus infection 1

Special Populations

• For patients being considered for allogeneic stem cell transplant, iron chelation therapy prior to transplant can decrease procedure-related hepatic complications 1
• Post-transplant phlebotomy is preferred for iron unloading in patients with favorable prognosis >1 year after stem cell transplant 1
• In patients with end-organ damage due to iron overload, regular phlebotomy should be performed to the same endpoints as standard treatment 1

Treatment Algorithm

1. Determine cause of hyperferremia:

   • Hereditary hemochromatosis
   • Secondary iron overload (transfusion-dependent)
   • Other causes

2. For hereditary hemochromatosis or primary iron overload:

   • Implement weekly phlebotomy (removing approximately 250 mg iron per session) 1
   • Continue until ferritin reaches 50-100 μg/L 1
   • Transition to maintenance phlebotomy (typically every 2-4 months) 1

3. For transfusion-dependent iron overload:

   • If ferritin >1,000 ng/mL and/or receiving ≥2 units blood/month for >1 year, initiate chelation therapy 1
   • Choose between deferoxamine (subcutaneous/IV) or oral chelators (deferasirox) based on patient factors 2, 3
   • Continue chelation as long as transfusions are needed 1

4. Monitor response:

   • Check ferritin levels every 3 months during treatment 1
   • Adjust frequency of phlebotomy or chelation dose based on response 1