This study was aimed to investigate the expression of CCAAT/enhancer binding protein alpha gene (c/ebpα) in patients with myelodysplastic syndromes (MDS) and to explore the significance of c/ebpα in pathogenesis and progression of MDS. Real time quantitative reverse transcriptase polymerase chain reaction (RQ-PCR) method was used to detect the expression level of c/ebpα mRNA in bone marrow mononuclear cells (BMMNC) of 33 patients with MDS and 14 normal controls. The results showed that the expression level of c/ebpα mRNA in low-risk and high-risk MDS was significantly lower than that of normal controls (p < 0.01, p < 0.001, respectively), moreover, high-risk MDS showed lower c/ebpα mRNA expression compared with low-risk MDS (p < 0.05). c/ebpα mRNA expression level in MDS was not correlated with sex, age and peripheral blood cell amount, while the ratio of blast cells in bone marrow was in the c/ebpα mRNA low expression group significantly higher than that in the high expression group (p < 0.01). It is concluded that down-regulation of c/ebpα mRNA expression level has closely associated with the pathogenesis of MDS, the c/ebpα may be an important molecular biological marker of MDS; the degree of down-regulated c/ebpα has closely related to the progression of MDS.