Treatment of High Iron Saturation
Therapeutic phlebotomy is the primary treatment for high iron saturation and iron overload, with the goal of reducing serum ferritin to 50-100 μg/L. 1
Diagnosis and Assessment
Before initiating treatment, proper assessment of iron overload is essential:
Primary monitoring tools:
- Serum ferritin - practical measure of body iron stores
- Transferrin saturation - ratio of serum iron to total iron-binding capacity 1
Secondary assessment methods (when needed):
- Liver biopsy - provides semiquantitative evaluation of iron excess
- MRI (T2*) - reflects tissue iron in vivo
- SQUID (Superconducting Quantum Interference Device) - quantitative measurement of liver iron 1
Treatment Algorithm
1. For Primary Hemochromatosis:
Phlebotomy Protocol:
- Remove 1 unit (450-500 mL) of blood weekly 1, 2
- Continue until serum ferritin reaches 10-20 μg/L
- Maintain ferritin at ≤50 μg/L with periodic phlebotomies 2
- Monitor hematocrit/hemoglobin before each phlebotomy to avoid reducing to <80% of starting value 1
- Check ferritin after every 10-12 phlebotomies (approximately every 3 months) 1
Maintenance Phase:
- Frequency varies by individual reaccumulation rate
- Some patients require monthly phlebotomy
- Others may need only 1-2 units removed per year 1
2. For Secondary Iron Overload:
For transfusion-dependent anemias (ineffective erythropoiesis):
- Iron chelation therapy is first-line treatment 1
- Options include:
For other secondary causes:
- Phlebotomy may be beneficial in:
- Porphyria cutanea tarda
- Chronic hepatitis C with iron overload
- Non-alcoholic fatty liver disease (NAFLD) 1
Special Considerations
Monitoring During Treatment:
- Assess serum ferritin every 3 months in transfusion-dependent patients 1
- Monitor organ function periodically 1
- For patients on chelation therapy, follow specific monitoring guidelines for the chelation product 1
Precautions:
- Avoid vitamin C supplementation during active iron reduction as it can accelerate iron mobilization and increase toxicity 1
- In patients with cardiac complications, initiate phlebotomy cautiously to prevent sudden death from rapid iron mobilization 1
- For patients with severe renal disease or anuria, deferoxamine is contraindicated 3
Potential Complications:
- Auditory and ocular toxicity with prolonged chelation therapy
- Renal toxicity
- Respiratory distress syndrome with high intravenous doses of chelators
- Growth suppression in pediatric patients 3
Expected Outcomes
With proper treatment:
- Resolution of hyperferritinemia
- Prevention of complications including hepatic cirrhosis, diabetes mellitus, joint disease, and cardiomyopathy
- Alleviation of symptoms such as weakness, fatigue, and hyperpigmentation 2
- Improved quality of life and increased longevity 2
Therapeutic phlebotomy remains the cornerstone of treatment for primary iron overload, while chelation therapy is essential for transfusion-dependent secondary iron overload. Early diagnosis and appropriate management are critical to prevent the serious complications of iron toxicity.