The effect of iron chelation on haemopoiesis in MDS patients with transfusional iron overload

The effect of iron chelation on haemopoiesis in MDS patients with transfusional iron overload
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DOI:
10.1046/j.1365-2141.1996.d01-1795.x
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发表时间:
1996-08-01
影响因子:
6.5
通讯作者:
Ellegaard, J
Ellegaard, J
中科院分区:
医学2区
文献类型:
--
作者:
Jensen, PD;Heickendorff, L;Ellegaard, J

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对11例骨髓增生异常综合征(MDS)患者在用去铁胺进行长达60个月的铁络合治疗期间的外周血细胞计数和Hb要求的变化进行了长期随访数据。通过重复测定血清转铁蛋白受体浓度来间接评价红系骨髓的活性。通过磁共振成像重复定量测定肝脏铁浓度来评价铁螯合的疗效,其中7/11(%)患者的Hb要求降低(大于或等于50%)。5名患者(46%)不再输血。7/11(%)患者血小板计数升高,7/9(78%)可评价患者中性粒细胞计数升高。所有铁螯合有效的患者都表现出红细胞生成量的改善并伴随着血清转铁蛋白受体浓度的增加。结论是,减少MDS患者的红细胞减少可以通过以下治疗实现:使用去铁胺,如果铁螯合有效并且患者接受了足够长的时间治疗,那么去铁胺的治疗效果如何仍然是一个进一步研究的挑战。
Long-term follow-up data are presented on changes in peripheral blood counts and Hb requirements of 11 patients with myelodysplastic syndromes (MDS) during iron chelation treatment with desferrioxamine for up to 60 months. The erythroid marrow activity was indirectly evaluated by repeated determinations of the serum transferrin receptor concentration. The efficacy of iron chelation was evaluated by repeated quantitative determination of the liver iron concentration by magnetic resonance imaging.Reduction in the Hb requirement (greater than or equal to 50%) was seen in 7/11 (64%) patients. Five patients (46%) became blood transfusion independent. Platelet counts increased in 7/11 (64%) patients and the neutrophil counts in 7/9 (78%) evaluable patients. All patients in whom iron chelation was highly effective showed improvement of erythropoietic output accompanied by an increase in the serum transferrin receptor concentration.It is concluded that reduction in cytopenia in MDS patients may be accomplished by treatment: with desferrioxamine, if the iron chelation is efficient and the patients are treated for a sufficiently long period of time, Exactly how treatment with desferrioxamine works remains a challenge for further investigation.