From the Guidelines
Phlebotomy should be performed weekly or fortnightly during the induction phase until iron stores are depleted, with a target serum ferritin of 50 lg/L, and then maintained in the range of 50-100 lg/L during the maintenance phase. This approach is based on the most recent guidelines from the Journal of Hepatology 1, which emphasize the importance of careful monitoring of serum ferritin and haemoglobin levels to avoid overtreatment and iron deficiency.
Key Considerations
- The volume and frequency of phlebotomies required to reach these goals are usually 400–500 ml weekly or every 2 weeks during the induction phase, and every 1–4 months during the maintenance phase, depending on the patient’s iron status 1.
- Serum haemoglobin should always be monitored during both induction and maintenance phases, and if haemoglobin concentrations are <12 g/dl, the frequency of phlebotomy should be decreased 1.
- Serum ferritin should be monitored every month during the induction phase, and every 6 months during the maintenance phase to adapt the treatment schedule and ensure that ferritin remains within the target range 1.
Monitoring and Adjustments
- Unexpected changes in serum ferritin or transferrin saturation levels should always be investigated, as significant fluctuations are not a feature of haemochromatosis 1.
- It is advisable to periodically check plasma folate and plasma cobalamin, especially in patients who require numerous venesections, and administer vitamin supplements if necessary 1.
From the Research
Phlebotomy Overview
- Phlebotomy is a therapy used to treat iron overload associated with hemochromatosis, a genetic disorder that can lead to end-organ failure and death if left untreated 2.
- The goal of phlebotomy is to reduce iron levels in the body by removing blood, which contains iron, from the body 3.
Patient Compliance with Phlebotomy
- A study published in 2003 found that 96.6% of patients with hemochromatosis achieved iron depletion through phlebotomy, and 33.1% and 43.2% followed weekly and biweekly schedules, respectively 3.
- However, the study also found that average compliance with maintenance therapy decreased by 6.8% annually, with a mean follow-up of 4.1 years after achieving iron depletion 3.
Risks and Complications of Phlebotomy
- Excessive therapeutic phlebotomy can lead to iron deficiency, which can cause symptoms such as anemia, hypochromia, and microcytosis 4.
- A study published in 2000 found that 13 adults with hemochromatosis developed iron deficiency due to excessive phlebotomy, which persisted for an average of 25 months before diagnosis 4.
Comparison of Phlebotomy with Other Treatments
- A study published in 2016 compared the effects of phlebotomy and erythrocytapheresis on iron parameters in patients with hereditary hemochromatosis, and found that erythrocytapheresis led to a more pronounced decrease in serum ferritin per treatment procedure, without a larger decrease in serum hepcidin 5.
Screening and Diagnosis
- A systematic review published in 2005 found that the prevalence of hereditary hemochromatosis in primary care patients was estimated to be between 1 in 169 and 1 in 556 patients, but the available evidence did not demonstrate that the benefits of screening outweigh the risks and costs 6.
- The review also found that a serum ferritin level less than 1000 microg/L was predictive of absence of cirrhosis, and that diagnostic studies varied with respect to case definition 6.