Dörmer P, Schalhorn A, Wilmanns W, Hershko C
Gesellschaft für Strahlen- und Umweltforschung, München, Federal Republic of Germany.
Br J Haematol. 1987 Sep;67(1):61-6. doi: 10.1111/j.1365-2141.1987.tb02297.x.
Patterns of erythropoiesis and granulopoiesis were studied in 15 patients with myelodysplastic syndromes and in one with smouldering leukaemia by correlating CFU-C, BFU-E and CFU-E cloning efficiency with erythroid and myeloid maturation indices derived from quantitative 14C-autoradiography and with ferrokinetics. Maturation index of a cell lineage was defined as the ratio of cell production rate increase from the first to the last proliferative compartment over the corresponding normal value. The myeloid maturation index was reduced in all cases, but CFU-C progenitor frequency was increased by a factor of 3. Erythroid maturation index was also reduced in most cases, and BFU-E progenitor frequency was reduced by a factor of 2. Similarly, CFU-E cloning efficiency corrected for the erythroid maturation index was 2.5-10-fold lower than normal. Comparison of the erythroid maturation index with ferrokinetics revealed a constant ratio of ineffective erythropoiesis with a maturation disturbance in the proliferative pool twice that in the non-proliferative pool. These findings indicate basic qualitative and quantitative abnormalities and a difference in the patterns of production of erythroid and myeloid cells in the myelodysplastic syndromes: Myeloid progenitors are increased in number but their maturation is grossly abnormal. On the other hand, the apparent reduction in erythroid progenitors and the reduced erythroid maturation index are attributable to both premature cell death of the more mature erythroblasts and an increased proerythroblast proliferation, i.e. extra divisions in the proerythroblast compartment at the expense of maturation.
通过将集落形成单位 - 粒细胞（CFU - C）、爆式红系集落形成单位（BFU - E）和红细胞集落形成单位（CFU - E）克隆效率与源自定量¹⁴C - 放射自显影的红系和髓系成熟指数以及铁动力学相关联，对15例骨髓增生异常综合征患者和1例冒烟型白血病患者的红细胞生成和粒细胞生成模式进行了研究。一个细胞系的成熟指数定义为从第一个到最后一个增殖区室细胞产生率增加的比率与相应正常值的比值。所有病例中髓系成熟指数均降低，但CFU - C祖细胞频率增加了3倍。大多数病例中红系成熟指数也降低，BFU - E祖细胞频率降低了2倍。同样，经红系成熟指数校正后的CFU - E克隆效率比正常低2.5至10倍。红系成熟指数与铁动力学的比较显示，无效红细胞生成与增殖池成熟障碍的恒定比率是非增殖池的两倍。这些发现表明骨髓增生异常综合征中红系和髓系细胞生成模式存在基本的定性和定量异常及差异：髓系祖细胞数量增加但其成熟严重异常。另一方面，红系祖细胞明显减少和红系成熟指数降低归因于更成熟的成红细胞过早死亡以及原红细胞增殖增加，即在原红细胞区室额外分裂而牺牲了成熟。
