Poullin P, Lefèvre P A
Service d'hémaphérèse et d'autotransfusion, hôpital de La-Conception, 147, boulevard Baille, 13385 Marseille cedex 05, France.
Transfus Clin Biol. 2011 Dec;18(5-6):553-8. doi: 10.1016/j.tracli.2011.04.004. Epub 2011 Oct 22.
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.
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%.
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.
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.
遗传性血色素沉着症(HH)患者通常建议采用每周放血计划以实现铁耗竭。然而,对于严重铁过载患者,可能需要两年多时间,这会导致疲劳和依从性差。十多年来,我们一直使用红细胞单采术(EA)作为替代治疗方法。
为评估实现铁耗竭所需的EA次数、铁耗竭治疗的持续时间以及耐受性,我们回顾性分析了2001年至2007年在我科随访的新诊断血色素沉着症患者的数据,这些患者为C282Y突变纯合子。使用间断或连续流动细胞分离器进行EA。方案为每两个月进行一次EA,直至血清铁蛋白正常化,目的是在每次治疗结束时将患者的血细胞比容降至32 - 35%。然后每月进行一次EA,直至完全去饱和,定义为血清铁蛋白浓度低于50μg/L且转铁蛋白饱和度低于40%。
纳入30例患者(23例男性,平均年龄52岁,范围25 - 78岁),分析了625次操作。每次操作去除的红细胞平均体积为416.4 mL(范围150 - 948),相当于去除374 mg铁。11个月内进行20次治疗(范围14 - 78)后实现铁耗竭(铁蛋白<50μg/L)。在单采过程中和之后未观察到严重不良反应或枸橼酸盐毒性。在铁耗竭治疗期间未报告有特定疲劳。患者依从性为100%。18例有症状患者中有12例临床症状改善。
我们得出结论,每两个月进行一次EA治疗的HH患者在耐受性良好的情况下不到1年即可实现铁耗竭。这些数据支持在严重铁过载患者中使用EA,因为它可能减少治疗次数以及铁耗竭治疗的持续时间。