Treatment & Management
Monitoring Treatment
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Initial De-ironing Phase
- Initial de-ironing usually takes from 3 months to 1 year.
- Volume of blood to be removed varies among patients.
- Typically, 1 unit (500 mL) of blood is removed per week.
- Patients with small body mass (i.e., women <110 lbs), elderly
patients, and patients with anemia, cardiac problems, or pulmonary
problems can often sustain removal of only 250 mL of blood per week.
- Initial iron depletion is complete when
- the serum ferritin level is between 25 and 50 ng/mL,
- the hemoglobin concentration is lower than 11.0 mg/dL, or
- the hematocrit is lower than 0.33 for more than 3 weeks (in patients
who do not have chronic anemia).
These values indicate that mild iron deficiency has been induced and
potentially pathogenic iron deposits have been removed.
Monitoring Treatment
Serum ferritin level is the most reliable, readily available, and
inexpensive way to monitor therapeutic phlebotomy.
In general,
patients who have higher serum ferritin levels have more severe iron
overload and need more phlebotomies.
- Among patients who have serum ferritin levels greater than 1000 ng/mL
before treatment, it is sufficient to quantify the serum ferritin level
every 4 to 8 weeks during the initial months of treatment.
- The serum ferritin levels should be measured more often in patients
who have received multiple phlebotomy treatments and in those who have
mild or moderate iron overload at diagnosis.
- Some experts also suggest checking hemoglobin values at each visit
during treatment, while others suggest monitoring every 4–6 weeks. Normal
hemoglobin levels range from 12–16 g/dL for females and 14–18 g/dL for
males (Merck Manual, 1999).
In all patients, serum ferritin levels should be quantified after each
additional one or two treatments once the value is less than or equal to 100 ng/mL.
Clinical judgment and careful monitoring are essential to treatment.
Preventing Abnormal Iron Reaccumulation
To prevent abnormal iron reaccumulation, serum ferritin levels should be
monitored and additional phlebotomies scheduled as warranted.
- For the first year, determining how often phlebotomy should be
performed is a matter of trial and error by physician and patient.
The table below depicts normal ranges and ideal maintenance for serum
ferritin for men and women.
| Serum Ferritin |
Adult Males |
Adult Females |
|
Normal range
|
25–300 ng/mL |
25–200 ng/mL |
|
Ideal maintenance |
25–50 ng/mL |
25–50 ng/mL |
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(CDC Expert Panel on Hemochromatosis, 2000 and 2002; Barton JC, 1998 and
2000) |
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Lifetime Maintenance
Continued lifetime monitoring is key to appropriate patient
management. Phlebotomy should be performed throughout a patient’s life to
keep ferritin levels between 25 and 50 ng/mL.
This generally requires the annual removal of 3 or 4 units in men and 1
or 2 units of blood in women, on average.
- Some persons, especially elderly persons, may not require maintenance
phlebotomies, but their serum ferritin levels should be monitored at least
once a year (Barton JC, 2000).
At a minimum, yearly serum ferritin checks for continued maintenance of
low body iron stores can prevent added hemochromatosis complications.
Careful monitoring of each patient throughout treatment is imperative. If
treatment is too aggressive, anemia may result.
The logic of phlebotomy treatment is to induce mild temporary anemia and
maintain it until iron storage is greatly reduced.
- When phlebotomy is too aggressive, however, red blood cell production
is not sufficient to sustain healthy hemoglobin level, and forced anemia
can result.
- Some physicians mistakenly use this initial state of anemia to signal
that the patient is de-ironed and treatment can be suspended.
Younger patients can tolerate a temporary state of mild anemia but older
patients may have difficulty with this approach to de-ironing. Extremely
young or old patients, as well as those who are small and frail, may require
an adjustment in the amount of blood removed.
After the initial de-ironing is accomplished, maintaining overt clinical
anemia by phlebotomy is not justifiable. After iron depletion, the
hemoglobin concentration and hematocrit should be allowed to return to and
remain within the normal range (Barton JC, 1998).
Patients with hemoglobin concentrations lower than 11 mg/dL or
hematocrits lower than 0.33 before treatment are more likely to have
symptoms of hypovolemia and anemia; phlebotomy is less efficient at removing
iron in these patients.
Many patients with chronic hemolytic anemia and iron overload, however,
DO NOT tolerate phlebotomy well (Barton JC, 2000).
Notes: Patients should be instructed to hydrate before and after a
phlebotomy to prevent hypovolemia complications. Fluid intake of
approximately 2 L per day is appropriate before and after the procedure.
Patients should be observed for signs of hypovolemia (e.g., hypotension,
tachycardia, increased respiratory rate, dizziness, weakness, change in
mental status) during a phlebotomy and directly after.
Elderly patients or patients with cardiac disease histories may require
closer monitoring because they may more easily develop signs of hypovolemia.
These patients also may need less blood removed at longer intervals (Wright
SM, 2000).
Additional monitoring considerations include hemoglobin concentration,
hematocrit, and mean corpuscular volume.
Clinical judgment and careful monitoring are essential to treatment.
Chelation Therapy
For patients who cannot be bled because of other heritable and acquired
anemias, chelation therapy is an option.
- Iron chelation is the pharmacological removal of metals by chemicals
that bind metal so that it is excreted in urine.
- The only pharmacological iron-chelating agent approved by the FDA for
use in humans is intravenous deferoxamine, aka desferrioxamine, (DfO,Desferal*).
This approach lacks the complete efficacy of phlebotomy and should be
employed only when absolutely necessary.
* Desferal™ must never be used with compazine; the combination produces
coma.
Iron chelation should not be confused with EDTA (ethylenediamine
tetra-acetic acid) chelation.
- Claims for dietary supplements and other over-the-counter products
that remove heavy metals may not have supporting data, especially for iron
overload treatment.
When to refer: A primary care physician can manage
hemochromatosis; however, if a patient has advanced disease, you may want to
refer the patient to the appropriate specialist.
Where to refer: Contacts for therapeutic phlebotomy include
- Hospital blood banks.
- Community blood banks.
- Plasma centers.
- IV therapy centers.
- Local county medical society.
A hematologist may also be able to help identify phlebotomy sources.
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