What are the common orders for phlebotomy in patients with hereditary hemochromatosis (HH)?

Phlebotomy Protocol for Hereditary Hemochromatosis

For patients with hereditary hemochromatosis, therapeutic phlebotomy should be performed weekly or biweekly (removal of 500 mL blood per session) during the initial iron depletion phase, followed by individualized maintenance phlebotomy to maintain serum ferritin between 50-100 μg/L. 1, 2

Initial Iron Depletion Phase

• Remove one unit of blood (approximately 500 mL) weekly or biweekly as tolerated, with each unit containing approximately 200-250 mg of iron 1, 2
• Check hemoglobin/hematocrit prior to each phlebotomy session 1
• Ensure hemoglobin/hematocrit does not fall below 80% of the starting value to prevent anemia 1
• Monitor serum ferritin level every 10-12 phlebotomies (approximately every 3 months) 1, 2
• Continue frequent phlebotomy until serum ferritin reaches 50-100 μg/L 1
• The initial depletion phase may take up to 2-3 years for patients with significant iron overload (>30g total body iron) 1, 3

Maintenance Phase

• Once target ferritin (50-100 μg/L) is reached, transition to maintenance phlebotomy 1, 2
• Frequency of maintenance phlebotomy varies significantly among individuals due to variable rates of iron reaccumulation 1, 2
• Some patients require monthly maintenance phlebotomy, while others may need only 1-2 units removed per year 1
• Monitor serum ferritin every 6 months to adjust treatment schedule 2
• Continue lifelong maintenance phlebotomy to keep ferritin between 50-100 μg/L 1

Monitoring Parameters

• Check hemoglobin/hematocrit before each phlebotomy 1
• If hemoglobin falls below 12 g/dL, decrease frequency of phlebotomy 2, 4
• Discontinue phlebotomy temporarily if hemoglobin falls below 11 g/dL 4
• Monitor serum ferritin to ensure target range is maintained and to avoid iron deficiency 1, 2

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, 4
• No dietary adjustments are necessary 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 acquired by therapeutic phlebotomy may be used for blood donation in some institutions, as approved by the American Red Cross and FDA 1
• Avoid raw shellfish due to risk of Vibrio vulnificus infection in patients with HH 1

Alternative Treatment Options

• Erythrocytapheresis (selective removal of erythrocytes) may be considered as an alternative to standard phlebotomy 5, 6
• Erythrocytapheresis can remove up to 1000 mL of erythrocytes per session (compared to 250 mL per phlebotomy), potentially reducing the number of treatment sessions by up to 70% 6, 7
• Consider erythrocytapheresis in patients who cannot tolerate frequent phlebotomy or require rapid iron depletion 5, 7

Common Pitfalls to Avoid

• Stopping treatment too early before reaching target ferritin levels 1, 2
• Failing to transition to maintenance therapy after initial iron depletion 1, 2
• Creating iron deficiency by excessive phlebotomy (target is iron normalization, not deficiency) 1, 2
• Neglecting to monitor hemoglobin/hematocrit before each phlebotomy 1
• Discontinuing maintenance therapy prematurely, as most patients require lifelong treatment 1, 6