Dr. Irene Pamler (Regensburg/ DE), Ivana Treneva (Regensburg/ DE), Dr. Andreas Michael Brosig (Regensburg/ DE), Dr. Morad Mohrez (Regensburg/ DE), Ikram Tlili (Regensburg/ DE), Adelina Florina Bica (Regensburg/ DE), PD Dr. Robert Offner (Regensburg/ DE), Prof. Dr. Ralph Burkhardt (Regensburg/ DE)
Background
The standard therapy for hereditary hemochromatosis (HH) is the removal of red blood cells to achieve serum ferritin target levels of <50µg/L. Phlebotomy as the classic gold standard and therapeutic erythrocytapheresis (TE) as a highly effective apheresis technique represent two possible treatment methods. We investigated the effectiveness of TE and analyzed the required number of treatment cycles in five patients with hemochromatosis.
Methods
Between 04/2020 and 04/2023, five patients with HH (39 to 66 years, body weight 85kg to 125kg)] were treated with TE (Spectra Optia, Terumo BCT, exchange set, erythrocyte depletion technique). One patient was newly diagnosed while 4 patients were pretreated and had undergone phlebotomy previously but required a switch to TE due to limited venous access. Erythrocyte volumes for depletion were set in proportion to the total blood volume (TBV) and according to the hematocrit: 205 to 793 mL of RBCs were withdrawn per single treatment procedure. Hemoglobin, hematocrit, and serum ferritin levels were analyzed before and after treatment. Depending on these results we established further TE intervals.
Results
We performed 75 TEs in 4 men and 1 woman. Prior to our treatment, serum ferritin levels ranged from 1614ng/mL to 152ng/mL, with lower serum ferritin levels measured in two of the pre-treated patients. The average number of required treatments to decrease serum ferritin levels below 50µl was 11, ranging from 3 to 19 with an average of 23 days between treatments. Hematocrit levels before treatment ranged from 31,8% to 49,1% and after treatment from 28,7% to 42,7%. After reaching a serum ferritin level of ≤50µg/L, another TE was required on average every 66 days.
Conclusion
All five patients with HH were successfully treated with TE and achieved optimal serum ferritin levels, although two had difficult venous conditions and low hematocrit limiting phlebotomy. Also for TE, a low hematocrit represents an important limiting factor that restricted TE frequency. While phlebotomy is more accessible for patients with HH, TE can provide the required serum ferritin levels in a short time interval in difficult clinical situations and is also well tolerated.
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