What is the treatment of hereditary hemochromatosis?
Hereditary hemochromatosis is a condition of systemic iron overload. First-line management is directed at reducing excess iron stores to a defined target, monitored through serum ferritin.
Clinical Goal
Treatment aims to bring serum ferritin down to between 50 and 100 µg/L, a level that indicates excess iron stores have been mobilised. Response is reassessed after approximately every 10–12 treatment sessions.
Treatment Approach
The structured first-line approach centres on a specific blood-removal procedure performed on a scheduled basis, with careful haematologic monitoring before each session. Full sequencing, session parameters, and monitoring criteria are in the complete protocol →
References
DOI: 10.1002/hep.24330
- Patients with hemochromatosis and iron overload should undergo therapeutic phlebotomy weekly (as tolerated).
- One unit of blood contains approximately 200–250 mg iron, depending on the hemoglobin concentration, and should be removed once or twice per week as tolerated.
- Each phlebotomy should be preceded by measurement of the hematocrit or hemoglobin so as to avoid reducing the hematocrit/hemoglobin to <80% of the starting value.
- Serum ferritin analysis should be performed after every 10–12 phlebotomies (approximately 3 months) in the initial stages of treatment.
- Phlebotomy can be stopped at the point at which iron stores are depleted, and the patient should be assessed for whether they require maintenance phlebotomy.
- During treatment for HH, dietary adjustments are unnecessary.
- Vitamin C supplements and iron supplements should be avoided.
- Target levels of phlebotomy should be a ferritin level of 50–100 µg/L.
- It can be confidently assumed that excess iron stores have been mobilized when the serum ferritin drops to between 50 and 100 µg/L.
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