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对中间型β地中海贫血患者给予高剂量重组人促红细胞生成素:一项初步试验。

Administration of high doses of recombinant human erythropoietin to patients with beta-thalassemia intermedia: a preliminary trial.

作者信息

Bourantas K, Economou G, Georgiou J

机构信息

Hematology Unit, Department of Internal Medicine, University of Ioannina Medical School, Greece.

出版信息

Eur J Haematol. 1997 Jan;58(1):22-5. doi: 10.1111/j.1600-0609.1997.tb01405.x.

DOI:10.1111/j.1600-0609.1997.tb01405.x
PMID:9020369
Abstract

Four patients (1 male, 3 female, age range 16-56 yr) with beta-thalassemia intermedia were given high doses of recombinant human erythropoietin (rHuEpo), iron sulfate and folic acid in an attempt to improve their anemia. The dose schedule was: rHuEpo, 500 U/kg 3 times weekly, iron sulfate, 300 mg/d and folic acid, 5 mg/d. All patients were red blood cell transfusion-dependent. Hematological data and fetal hemoglobin (HbF) were assayed every 2 wk. XmnI polymorphism and beta-thalassemia mutations were identified by PCR. All patients showed a moderate to high increase in hemoglobin values (mean value: 2.5 g/dl) and in 1 patient HbF levels also increased; 3 patients became red blood cell transfusion-independent and 1 patient was able to extend the intervals between transfusions significantly. No side effects were observed during rHuEpo therapy.

摘要

对4例中间型β地中海贫血患者(1例男性,3例女性,年龄范围16 - 56岁)给予大剂量重组人促红细胞生成素(rHuEpo)、硫酸亚铁和叶酸,以改善其贫血状况。给药方案为:rHuEpo,500 U/kg,每周3次;硫酸亚铁,300 mg/d;叶酸,5 mg/d。所有患者均依赖红细胞输血。每2周检测血液学数据和胎儿血红蛋白(HbF)。通过聚合酶链反应(PCR)鉴定XmnI多态性和β地中海贫血突变。所有患者血红蛋白值均有中度到高度升高(平均值:2.5 g/dl),1例患者的HbF水平也有所升高;3例患者不再依赖红细胞输血,1例患者能够显著延长输血间隔时间。在rHuEpo治疗期间未观察到副作用。

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