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重度β地中海贫血多次输血患者血清促红细胞生成素对贫血的反应迟钝。

Blunted serum erythropoietin response to anemia in patients polytransfused for beta-thalassemia major.

作者信息

Chen J S, Lin K H, Wang S T, Tsao C J, Yeh T F

机构信息

Department of Pediatrics, National Cheng Kung University Hospital, Taipei, Taiwan.

出版信息

J Pediatr Hematol Oncol. 1998 Mar-Apr;20(2):140-4. doi: 10.1097/00043426-199803000-00010.

Abstract

PURPOSE

To investigate the response of erythropoietin (EPO) to anemia in patients polytransfused for beta-thalassemia major.

PATIENTS AND METHODS

We measured the serum EPO levels and the concurrent hemoglobin (Hb) concentrations in 40 patients polytransfused for beta-thalassemia major, in 18 patients with iron deficiency anemia (IDA), and 32 healthy subjects. Serum EPO levels were assayed by an enzyme immunoassay.

RESULTS

In both groups with beta-thalassemia major and IDA, serum EPO levels were significantly elevated (114 +/- 71 and 239 +/- 217 mU/mL, respectively). There was a significant inverse correlation between log EPO values and Hb concentrations in patients with beta-thalassemia major (r = 0.61; p < 0.01) and IDA (r = 0.81; p < 0.01). In a semilogarithmic plot, the slope of the regression line obtained in patients with beta-thalassemia major was significantly lower than that of IDA (p < 0.01), suggesting a blunted EPO response to anemia in patients polytransfused for beta-thalassemia major. The elevation of serum EPO in patients with beta-thalassemia major was poorly related to clinical variables except serum ferritin.

CONCLUSIONS

We conclude that a significant inverse relationship between serum EPO levels and Hb concentration exists in patients with beta-thalassemia major. However, this EPO response in patients with anemia caused by beta-thalassemia major may be blunted when compared to patients with IDA.

摘要

目的

研究重型β地中海贫血多次输血患者促红细胞生成素(EPO)对贫血的反应。

患者与方法

我们检测了40例重型β地中海贫血多次输血患者、18例缺铁性贫血(IDA)患者和32名健康受试者的血清EPO水平及同期血红蛋白(Hb)浓度。采用酶免疫测定法检测血清EPO水平。

结果

重型β地中海贫血组和IDA组患者的血清EPO水平均显著升高(分别为114±71和239±217 mU/mL)。重型β地中海贫血患者和IDA患者的log EPO值与Hb浓度之间均存在显著负相关(r分别为0.61;p<0.01和r为0.81;p<0.01)。在半对数图中,重型β地中海贫血患者所得回归线的斜率显著低于IDA患者(p<0.01),提示重型β地中海贫血多次输血患者对贫血的EPO反应迟钝。重型β地中海贫血患者血清EPO的升高与除血清铁蛋白外的临床变量关系不大。

结论

我们得出结论,重型β地中海贫血患者血清EPO水平与Hb浓度之间存在显著负相关。然而,与IDA患者相比,重型β地中海贫血所致贫血患者的这种EPO反应可能迟钝。

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