Dan K, An E, Futaki M, Inokuchi K, Gomi S, Yamada T, Ogata K, Tanabe Y, Ohki I, Shinohara T
Third Department of Internal Medicine, Nippon Medical School, Tokyo, Japan.
Acta Haematol. 1993;89(3):113-8. doi: 10.1159/000204502.
Bone marrow progenitor cell assays of three cell lineages, i.e., colony-forming unit megakaryocytes (CFU-Meg), burst-forming unit erythrocytes (BFU-E) and colony-forming unit granulocyte-macrophages (CFU-GM), were performed for 21 patients with myelodysplastic syndromes (MDS). Markedly reduced or absent colony formation was found in 67% of the patients for CFU-Meg and all patients except 2 with refractory anemia (RA) for BFU-E. Abnormal CFU-GM colony formation was found in only 5 of 12 patients with RA and RA with ring sideroblasts, in contrast to all of the RA patients with excess of blasts and excess of blasts in transformation. Defective colony formation of all three cell lineages was seen in 63% of the MDS patients. The colony number of CFU-Meg correlated significantly with the numbers of both BFU-E and CFU-GM. These findings indicate that hematopoiesis in MDS patients is disturbed due to a qualitative or quantitative defect at the multipotent stem cell level.
对21例骨髓增生异常综合征(MDS)患者进行了三种细胞系的骨髓祖细胞检测,即巨核细胞集落形成单位(CFU-Meg)、红细胞爆式集落形成单位(BFU-E)和粒-巨噬细胞集落形成单位(CFU-GM)。67%的患者CFU-Meg集落形成明显减少或缺失,除2例难治性贫血(RA)患者外,所有患者的BFU-E均如此。仅12例RA和环状铁粒幼细胞性RA患者中的5例发现CFU-GM集落形成异常,相比之下,所有伴有原始细胞增多的RA患者和转化中的原始细胞增多患者均如此。63%的MDS患者三种细胞系集落形成均有缺陷。CFU-Meg的集落数与BFU-E和CFU-GM的数量均显著相关。这些发现表明,MDS患者的造血功能因多能干细胞水平的质量或数量缺陷而受到干扰。