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

Treatment of Hyperferremia (High Iron Levels)

The primary treatment for hyperferremia is therapeutic phlebotomy, which should be performed weekly with removal of 400-500 ml of blood until ferritin levels reach 50-100 μg/L, followed by maintenance phlebotomies every 1-4 months to maintain these target levels. 1

Diagnostic Evaluation Before Treatment

Before initiating treatment, it's important to:

• Measure serum ferritin and transferrin saturation (TSAT) to confirm iron overload
• Consider liver biopsy if ferritin levels are >1000 μg/L with elevated liver enzymes 2, 1
• Evaluate for underlying causes (hereditary hemochromatosis, transfusional iron overload, etc.)
• Assess for complications of iron overload (liver disease, cardiac dysfunction, diabetes)

Treatment Algorithm

First-line Treatment: Therapeutic Phlebotomy

For patients with hereditary hemochromatosis or other forms of iron overload who can tolerate phlebotomy:

1. Initial Phase:

   • Remove 400-500 mL of blood weekly 2, 1
   • Check hematocrit/hemoglobin before each procedure to ensure it doesn't decrease by more than 20% from previous value 2
   • Monitor ferritin levels every 10-12 phlebotomies 2

2. Maintenance Phase:

   • Begin when ferritin levels reach 50-100 μg/L 1
   • Continue phlebotomy every 1-4 months to maintain ferritin between 50-100 μg/L 2, 1
   • For elderly patients, more relaxed ferritin targets (200-300 μg/L) may be appropriate 1

Second-line Treatment: Iron Chelation Therapy

For patients who cannot tolerate phlebotomy or have transfusion-dependent anemias:

1. Deferoxamine (Desferal):

   • Administered subcutaneously or intravenously at 20-40 mg/kg/day 2
   • Used primarily for secondary iron overload due to dyserythropoiesis 2

2. Deferasirox (oral):

   • Dosing based on baseline liver iron concentration (LIC): 5-30 mg/kg/day 3
   • Effective in reducing LIC and serum ferritin levels 3
   • Monitor renal function, liver function, and blood counts during therapy 1, 3

Monitoring During Treatment

• Ferritin levels: Check regularly to guide therapy
• Complete blood count: Before each phlebotomy
• Liver function tests: Regular monitoring
• Folate and vitamin B12 levels: Monitor if numerous phlebotomies are required 1
• For patients with cirrhosis: Regular screening for hepatocellular carcinoma 2, 1

Lifestyle Modifications

• Avoid vitamin C supplements (>500 mg/day) as they may increase iron absorption and mobilization 2, 1
• Avoid iron supplements and iron-fortified foods 1
• Limit red meat consumption and alcohol intake 1
• Avoid raw shellfish due to risk of infections in iron-overloaded patients 1

Special Considerations

• Cardiac involvement: Use caution with rapid iron mobilization due to risk of arrhythmias 1
• Cirrhosis: Continue surveillance for hepatocellular carcinoma even after adequate iron depletion, as HCC accounts for approximately 30% of hemochromatosis-related deaths 2
• Treatment adherence: Emphasize the importance of long-term adherence, as early and consistent treatment significantly reduces morbidity and mortality 1

Pitfalls and Caveats

• Don't rely on total iron-binding capacity (TIBC) alone for treatment decisions in acute iron poisoning, as it can be artificially elevated 4
• Avoid vitamin C supplementation during phlebotomy or chelation therapy as it can accelerate iron mobilization and potentially increase toxicity 2, 1
• Don't discontinue monitoring after initial iron depletion; lifelong maintenance therapy is typically required 2
• Be aware that certain symptoms (arthropathy, hypogonadism, advanced cirrhosis) may not improve with iron removal 2