Aul C, Arning M, Runde V, Schneider W
Department of Internal Medicine, Heinrich Heine University, Düsseldorf, F.R.G.
Leuk Res. 1991;15(7):571-5. doi: 10.1016/0145-2126(91)90025-o.
Medullary dyserythropoiesis with reduced production of erythrocytes is an early and consistent feature of myelodysplastic syndromes (MDS). The mechanism underlying the disturbed red cell proliferation and maturation is presently unknown. In order to study the role of erythropoietic growth factors, we determined by radioimmunoassay the serum concentrations of immunoreactive erythropoietin (Epo) in 42 non-transfused patients with primary and secondary MDS. Their median hemoglobin concentration at the time of Epo measurement was 9.1 g/dl (range, 5.7-14.6). Compared with the control group, 83% of the MDS patients had increased serum Epo levels, ranging from 26-4530 mU/ml. Although in the entire patient population an inverse relationship between serum Epo and hemoglobin concentrations was noted (r = -0.35; p = 0.02), Epo titers differed markedly between patients at comparable degrees of anemia. In 7 patients presenting with a hemoglobin concentration between 5.9 and 11.9 g/dl, excessive elevations of Epo levels (greater than 500 mU/ml) were found. In contrast to previous observations, serum Epo concentrations were not shown to correlate with the percentage of erythroblasts in the bone marrow. There was, however, a significant relationship between the Epo activity and the degree of medullary dyserythropoiesis, as assessed by morphological criteria (p less than 0.01). From these data we conclude that the anemia in MDS is not due to an endogenous Epo deficiency. The marked variability of Epo production in these disorders is not fully explained by the degree of anemia, but may also reflect inherent abnormalities of the myelodysplastic erythropoiesis.
骨髓红细胞生成异常伴红细胞生成减少是骨髓增生异常综合征（MDS）早期且持续存在的特征。目前尚不清楚红细胞增殖和成熟紊乱的潜在机制。为了研究促红细胞生成生长因子的作用，我们通过放射免疫测定法测定了42例未输血的原发性和继发性MDS患者血清中免疫反应性促红细胞生成素（Epo）的浓度。在测定Epo时，他们的血红蛋白浓度中位数为9.1 g/dl（范围为5.7 - 14.6）。与对照组相比，83%的MDS患者血清Epo水平升高，范围为26 - 4530 mU/ml。尽管在整个患者群体中发现血清Epo与血红蛋白浓度呈负相关（r = -0.35；p = 0.02），但在贫血程度相当的患者中，Epo滴度差异显著。在7例血红蛋白浓度在5.9至11.9 g/dl之间的患者中，发现Epo水平过度升高（大于500 mU/ml）。与先前的观察结果相反，血清Epo浓度与骨髓中幼红细胞的百分比无相关性。然而，根据形态学标准评估，Epo活性与骨髓红细胞生成异常程度之间存在显著关系（p < 0.01）。从这些数据我们得出结论，MDS中的贫血并非由于内源性Epo缺乏所致。这些疾病中Epo产生的显著变异性不能完全用贫血程度来解释，还可能反映了骨髓增生异常性红细胞生成的内在异常。
