[Effectiveness of therapeutic erythrocytapheresis to achieve iron depletion in hereditary type 1 hemochromatosis: report of 30 cases].
OBJECTIVE
Weekly phlebotomy schedule is commonly recommended to achieve iron depletion in hereditary hemochromatosis (HH). However, in patients with severe iron overload, more than 2 years may be required, leading to fatigue and lack of compliance. For more than 10 years, we have used erythrocytapheresis (EA) as an alternative treatment.


METHODS
To assess the number of EA to achieve iron depletion and the duration of the iron depletion therapy as well of the tolerance, we retrospectively analysed the data of newly diagnosed hemochromatosis patients, homozygote for the C282Y mutation, followed in our department between 2001 and 2007. EA were performed using a discontinuous or a continuous flow cell separators. The protocol consisted in a bimonthly EA until normalisation of the serum ferritin, with the aim of reducing the patient's hematocrit between 32-35% at the end of each session. Then we performed monthly EA until complete desaturation, defined as serum ferritin concentration below 50 μg/L and transferrin saturation below 40%.


RESULTS
Thirty patients were included (23 male, mean age 52 years, range 25-78) and 625 procedures analyzed. The mean volume of removed erythrocytes in each procedure was 416.4 mL (range 150-948), which equals to 374 mg of removed iron. Iron depletion (ferritin < 50 μg/L) was achieved after 11 months with 20 sessions (range 14-78). No serious adverse reactions or citrate toxicity were observed during and after the apheresis procedures. No specific fatigue was reported during the iron depletion therapy. Patient compliance was 100%. Clinical improvement was noted in 12 out of 18 of symptomatic patients.


CONCLUSIONS
We conclude that HH patients treated with bimonthly EA achieved iron depletion in less than 1 year under good condition of tolerance. These data support the use of EA in patients with a severe iron overload, since it may reduce the number of the procedures as well as the duration of the iron depletion therapy.
Service d'hémaphérèse et d'autotransfusion, hôpital de La-Conception, 147, boulevard Baille, 13385 Marseille cedex 05, France. pascale.poullin@ap-hm.fr
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Lefèvre
D000328:Adult; D000368:Aged; D016238:Cytapheresis / Q000295:instrumentation / Q000379:methods; D004912:Erythrocytes; D005260:Female; D005293:Ferritins / Q000097:blood; D005434:Flow Cytometry; D006400:Hematocrit; D006432:Hemochromatosis / Q000235:genetics / Q000628:therapy*; D000071020:Hemochromatosis Protein; D015395:Histocompatibility Antigens Class I / Q000235:genetics; D006801:Humans; D007501:Iron / Q000097:blood*; D008297:Male; D008565:Membrane Proteins / Q000235:genetics; D008875:Middle Aged; D020125:Mutation, Missense; D010349:Patient Compliance; D017354:Point Mutation; D012189:Retrospective Studies
C103673:HFE protein, human; D000071020:Hemochromatosis Protein; D015395:Histocompatibility Antigens Class I; D008565:Membrane Proteins; D005293:Ferritins; D007501:Iron