What is the recommended frequency of blood donations for a patient with hemochromatosis (iron overload disorder)?

Frequency of Blood Donations in Hemochromatosis

For patients with hemochromatosis, phlebotomy should be performed weekly or biweekly (every 2 weeks) during the initial iron depletion phase, followed by individualized maintenance phlebotomy every 1-4 months to maintain serum ferritin between 50-100 μg/L. 1

Initial Iron Depletion Phase

• One unit of blood (approximately 500 mL) should be removed weekly or biweekly as tolerated, with each unit containing approximately 200-250 mg of iron 1
• Hemoglobin or hematocrit should be checked before each phlebotomy to ensure it does not fall below 80% of the starting value 1
• Serum ferritin should be monitored every 10-12 phlebotomies (approximately every 3 months) during the initial stages 1
• As ferritin approaches the target range (50-100 μg/L), testing should be performed more frequently to avoid iron deficiency 1
• The initial depletion phase may take up to 2-3 years for patients with significant iron overload (>30g of total body iron) 1

Maintenance Phase

• Once serum ferritin reaches 50-100 μg/L, the frequency of phlebotomy should be adjusted to maintain ferritin within this target range 1
• The frequency of maintenance phlebotomy varies significantly among individuals due to variable rates of iron reaccumulation 1:
  • Some patients require monthly phlebotomy 1
  • Others may need only 1-2 units removed per year 1
• On average, serum ferritin rises by approximately 100 μg/L per year without treatment 1
• During maintenance, serum ferritin should be monitored every 6 months to adjust the treatment schedule 1

Monitoring Parameters

• Hemoglobin/hematocrit should be checked before each phlebotomy 1
• If hemoglobin falls below 12 g/dL, the frequency of phlebotomy should be decreased 1
• If hemoglobin falls below 11 g/dL, phlebotomy should be discontinued temporarily 1
• Serum ferritin should be monitored to ensure the target range is maintained and to avoid iron deficiency 1

Special Considerations

• Avoid vitamin C supplements in iron-loaded patients, particularly during phlebotomy treatment, as they can accelerate iron mobilization to potentially dangerous levels 1
• Dietary modifications are generally unnecessary as the amount of iron absorption affected by a low-iron diet is small (2-4 mg/day) compared to the amount removed by phlebotomy (200-250 mg/unit) 1
• Patients with cardiac arrhythmias or cardiomyopathy require careful monitoring due to increased risk of sudden death with rapid iron mobilization 1
• Blood from hemochromatosis patients without complications can potentially be used for blood donation in some institutions 1, 2

Potential Complications

• Excessive phlebotomy can lead to symptomatic iron deficiency, which should be avoided 1, 3
• Signs of iron deficiency include fatigue, which has been reported after 25% of phlebotomies 4, 3
• Anemia may occur in up to 48% of patients undergoing frequent phlebotomy 4, 3
• Monitoring hemoglobin levels and serum ferritin is essential to prevent sustained iron deficiency 3, 5

By following this phlebotomy schedule and monitoring protocol, patients with hemochromatosis can effectively manage their iron levels and reduce the risk of complications associated with iron overload.