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妊娠期间,重组人促红细胞生成素诱导杂合子β地中海贫血贫血患者胎儿血红蛋白合成。

Induction of fetal hemoglobin synthesis with recombinant human erythropoietin in anemic patients with heterozygous beta-thalassemia during pregnancy.

作者信息

Breymann C, Fibach E, Visca E, Huettner C, Huch A, Huch R

机构信息

Department of Gynaecology and Obstetrics, Clinic of Obstetrics, University of Zurich, Switzerland.

出版信息

J Matern Fetal Med. 1999 Jan-Feb;8(1):1-7. doi: 10.1002/(SICI)1520-6661(199901/02)8:1<1::AID-MFM1>3.0.CO;2-O.

Abstract

OBJECTIVE

Recombinant human erythropoietin (rhEPO) increases fetal hemoglobin synthesis in nonpregnant thalassaemic patients. We used rhEPO in 4 pregnant patients with heterozygous beta-thalassemia and anemia to study its effect on erythropoiesis, F cell production, and HbF synthesis.

METHODS

Patients were treated with a combination therapy of rhEPO and iron. The effect on HbF synthesis was assessed by the percentage of F reticulocytes, F cells, and total HbF, erythropietis by reticulocyte count, and hemoglobin measurements and iron status by ferritin levels, transferrin saturation, and percentage of hypochromic red cells.

RESULTS

RhEPO caused an increase of F reticulocytes (1.5 to 10.5 fold), F cells (5.0 to 7.7 fold), and HbF (1.4 to 2.2 fold). All patients showed an increase of young, immature reticulocytes and had elevated reticulocytes at the end of therapy. Hemoglobin increased with a range from 0.3 to 1.5 g/dL. Transferrin saturation and ferritin levels were normal at the end of the study. There was an increase of the percentage of hypochromic red cells, indicating functional iron deficiency after rhEPO administration despite supplemental iron.

CONCLUSIONS

RhEPO stimulates both HbF synthesis and erythropoiesis in pregnant patients with heterozygous beta-thalassemia and anemia. Since it is known that high HbF levels ameliorate thalassemia symptoms in nonpregnant patients, use of rhEPO for the treatment of severe anemia in thalassaemic patients during pregnancy might be further evaluated.

摘要

目的

重组人促红细胞生成素(rhEPO)可增加非妊娠地中海贫血患者的胎儿血红蛋白合成。我们对4例患有杂合子β地中海贫血和贫血的孕妇使用rhEPO,以研究其对红细胞生成、F细胞产生及HbF合成的影响。

方法

患者接受rhEPO与铁剂的联合治疗。通过F网织红细胞、F细胞及总HbF的百分比评估对HbF合成的影响,通过网织红细胞计数评估红细胞生成,通过铁蛋白水平、转铁蛋白饱和度及低色素红细胞百分比评估血红蛋白测量及铁状态。

结果

rhEPO使F网织红细胞增加(1.5至10.5倍)、F细胞增加(5.0至7.7倍)及HbF增加(1.4至2.2倍)。所有患者年轻、未成熟网织红细胞均增加,且治疗结束时网织红细胞升高。血红蛋白增加范围为0.3至1.5 g/dL。研究结束时转铁蛋白饱和度及铁蛋白水平正常。低色素红细胞百分比增加,表明尽管补充了铁剂,但rhEPO给药后仍存在功能性缺铁。

结论

rhEPO可刺激患有杂合子β地中海贫血和贫血的孕妇的HbF合成及红细胞生成。鉴于已知高HbF水平可改善非妊娠患者的地中海贫血症状,rhEPO用于治疗妊娠期地中海贫血患者的严重贫血可能值得进一步评估。

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