Chen J S, Lin K H, Wang S T, Tsao C J, Yeh T F
Department of Pediatrics, National Cheng Kung University Hospital, Taipei, Taiwan.
J Pediatr Hematol Oncol. 1998 Mar-Apr;20(2):140-4. doi: 10.1097/00043426-199803000-00010.
To investigate the response of erythropoietin (EPO) to anemia in patients polytransfused for beta-thalassemia major.
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.
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.
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.
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。
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。
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。
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。
