Treatment
����Perform therapeutic phlebotomy weekly (as tolerated) on patients with
hemochromatosis and iron overload. (1A) Target levels for phlebotomy
should be a ferritin level of 50-100 mcg/L. (1B)
����In the absence of indicators suggestive of significant liver disease (ALT,
AST elevation), C282Y homozygotes who have an elevated ferritin (but
< 1000 mcg/L) should proceed to phlebotomy without a liver biopsy. (1B)
����Patients with end-organ damage due to iron overload should undergo
regular phlebotomy to the same endpoints as indicated above. (1A)
����During treatment for HH, dietary adjustments are unnecessary. Vitamin C
supplements and iron supplements should be avoided. (1C)
����Patients with hemochromatosis and iron overload should be monitored
for reaccumulation of iron and undergo maintenance phlebotomy. (1A)
Target levels of phlebotomy should be a ferritin level of 50-100 mcg/L.
(1B)
����Treat with phlebotomy patients with non-HFE iron overload who have an
elevated hepatic iron concentration (HIC) (1B)
����Perform iron chelation with either deferoxamine mesylate or deferasirox
in iron overloaded patients with dyserythropoietic syndromes or chronic
hemolytic anemia. (1A)
Table 5. Treatment of Hemochromatosis
Hereditary hemochromatosis
One phlebotomy (removal of 500 mL blood) weekly or biweekly
Check hematocrit/hemoglobin prior to each phlebotomy. Allow hematocrit/hemoglobin
to fall by no more than 20% of prior level
Check serum ferritin level every 10-12 phlebotomies
Stop frequent phlebotomy when serum ferritin reaches 50-100 mcg/L
Continue phlebotomy at intervals to keep serum ferritin between 50 and 100 mcg/L
Avoid vitamin C supplements
Secondary iron overload due to dyserythropoiesis
Deferoxamine (Desferal ��) at a dose of 20-40 mg/kg body weight per day IV, IM or SC
infusion
Deferasirox (Exjade��) given orally
Consider follow-up liver biopsy to ascertain adequacy of iron removal
Avoid vitamin C supplements
