Therapeutic erythrocytapheresis in the initial treatment of hereditary hemochromatosis

Acta Medica (Hradec Kralove). 2012;55(4):180-5. doi: 10.14712/18059694.2015.43.

Abstract

Background: The current treatment of hereditary hemochromatosis (HH) consists of performing periodic whole blood phlebotomies. Erythrocytapheresis (EA) can remove up to three times more red blood cells per single procedure and could thus have a clinical benefit. A prospective study of 30 consecutive cases of HH were included in a periodic EA program.

Methods and patients: EA were performed using a discontinuous flow cell separators. The protocol consisted of a bimonthly EA until normalization of the serum ferritin was reached. The aim was to reduce the total erythrocyte volume by 25-35%, eventually, to adjust the amount so that hematocrit would not drop below 0.25.

Results: 530 +/- 101 ml of erythrocytes were removed (median 517, range 116-761 ml). Iron depletion (ferritin < 20 microg/l) was achieved in all patients after a mean 6.9 +/- 7.6 months, median 5 months, range 1-36 months and a mean 14 EA sessions. The procedures were well tolerated and there were no severe side-effects.

Conclusions: We conclude that HH patients treated with EA achieved iron depletion quickly under good conditions of tolerance. The efficacy, speed, tolerability, and more favorable schedule of an EA program facilitate treatment of HH.

MeSH terms

  • Adult
  • Aged
  • Blood Component Removal*
  • Erythrocyte Transfusion*
  • Erythrocyte Volume
  • Female
  • Hematocrit
  • Hemochromatosis / blood
  • Hemochromatosis / genetics
  • Hemochromatosis / therapy*
  • Humans
  • Iron / blood
  • Male
  • Middle Aged
  • Transferrin / analysis

Substances

  • Transferrin
  • Iron