BACKGROUND: A multitude of recommendations exist for laboratory assays to m
onitor the pace and endpoints of phlebotomy therapy for hemochromatosis. Al
l of these recommendations rely on an assessment of storage iron to guide t
reatment, and none have been prospectively evaluated.
STUDY DESIGN AND METHODS: Nine consecutive patients underwent serial monito
ring of Hb, MCV, transferrin saturation, and ferritin during weekly phlebot
omy to deplete iron stores (induction therapy) and less frequent sessions t
o prevent iron reaccumulation (maintenance therapy). Changes in MCV and Hb
were used to guide the pace of phlebotomy over a median of 7 years of follo
w-up.
RESULTS: During induction therapy, the MCV increased transiently because of
reticulocytosis and then stabilized for a prolonged period before decreasi
ng more sharply which reflected iron-limited erythropoiesis. Iron depletion
was achieved after a median of 38 phlebotomies and removal of 9.0 g of iro
n. Maintenance phlebotomy was targeted to maintain the MCV at 5 to 10 perce
nt below prephlebotomy values and the Hb at > 13 g per dL. Transferrin satu
ration fluctuated considerably during treatment, but remained below 35 perc
ent during MCV-guided maintenance therapy. Ferritin values were not useful
guides to the pace of phlebotomy. The median maintenance therapy phlebotomy
interval was 7.5 weeks (range, 6-16), which corresponded to an average dai
ly iron removal of 35 to 67 mug per kg. Most patients showed evidence of ir
on reaccumulation at phlebotomy intervals of 8 weeks or more.
CONCLUSION: The MCV is an inexpensive, precise, physiologic indicator of er
ythropoietic iron availability. When used in conjunction with the Hb, it is
a clinically useful guide to the pace of phlebotomy therapy for hemochroma
tosis.