Therapeutic Erythrocytapheresis in the Initial Treatment of Hereditary Hemochromatosis

Vít Řeháček,Hana Jiroušová,Petr Papoušek,Milan Bláha,Jitka Černohorská

doi:10.14712/18059694.2015.43

Vít Řeháček, Hana Jiroušová + Show 3 more

Open Access

https://doi.org/10.14712/18059694.2015.43

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Abstract

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. 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. 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. 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.

Highlights

Hereditary hemochromatosis (HH) remains the most common, identified genetic disorder in Caucasians
Because withdrawal of blood is the most effective way to reduce iron storage in the body, the preferred treatment for iron overload associated with hemochromatosis is therapeutic phlebotomy [1, 4, 5, 26]
By means of EA at least 400 ml of red blood cells (RBC) can be collected at once which reduces the number of treatments

Summary

Introduction

Hereditary hemochromatosis (HH) remains the most common, identified genetic disorder in Caucasians. Because withdrawal of blood is the most effective way to reduce iron storage in the body, the preferred treatment for iron overload associated with hemochromatosis is therapeutic phlebotomy [1, 4, 5, 26]. This causes iron mobilization for incorporation into hemoglobin (Hb) during erythropoiesis [18]. By means of EA at least 400 ml of red blood cells (RBC) can be collected at once (but twice as this amount may be removed) which reduces the number of treatments. In this study we inform of the results with EA in HH therapy using our scheme and our modification under our conditions

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Conclusion