Department of Haematology, Sixth Hospital affiliated to Shanghai Jiaotong University, Shanghai, China.
Br J Haematol. 2010 May;149(4):587-97. doi: 10.1111/j.1365-2141.2010.08146.x. Epub 2010 Mar 16.
In the myelodysplastic syndromes (MDS), the haematopoietic cells show various levels of abnormal maturation and differentiation, which can be detected by flow cytometry. Testing the anomalies of stage- or lineage-specific surface antigens in CD34+ blasts can distinguish MDS from non-clonal cytopenic diseases, and also reflect the pathological characteristics of MDS as a class of clonal diseases for providing new clues to basic research. The present study established a flow cytometric scoring system (FCMSS) based on theproportion and antigenic co-expression of CD34+ blasts. This FCMSS showed good sensitivity and specificity (77.8% and 100%) in the assisted diagnosis of low-risk MDS without chromosome anomalies, ringed sideroblasts and excess marrow blasts. Moreover, we explored and reported different modes of abnormal expression of CD34+ blasts antigens in different disease stages and analyzed the biological significance of the immunotypes for the first time. We found expression of mature myeloid antigens and lymphoid antigens gradually decreased, and early functional antigens gradually increased from low-risk MDS with normal karytype to low-risk MDS with abnormal karyotype then to high-risk MDS. The patients with higher FCM scores were generally accompanied with HLA-DR15 allele or hypocellular marrow. Evolution of clones and immunological factors might have influence on expression of antigens in CD34+ blasts.
在骨髓增生异常综合征(MDS)中,造血细胞表现出不同程度的异常成熟和分化,可通过流式细胞术检测到。检测 CD34+ blasts 中阶段或谱系特异性表面抗原的异常可以将 MDS 与非克隆性细胞减少性疾病区分开来,并且还反映了 MDS 作为一类克隆性疾病的病理特征,为基础研究提供了新的线索。本研究建立了一种基于 CD34+blasts 比例和抗原共表达的流式细胞术评分系统(FCMSS)。该 FCMSS 在辅助诊断无染色体异常、环形铁幼粒细胞和过多骨髓blasts 的低危 MDS 时具有良好的灵敏度和特异性(77.8%和 100%)。此外,我们还首次探索并报告了不同疾病阶段 CD34+blasts 抗原异常的不同表达模式,并分析了免疫表型的生物学意义。我们发现,从核型正常的低危 MDS 到核型异常的低危 MDS 再到高危 MDS,成熟髓系抗原和淋巴系抗原的表达逐渐减少,而早期功能抗原逐渐增加。FCM 评分较高的患者通常伴有 HLA-DR15 等位基因或低细胞骨髓。克隆的进化和免疫因素可能会影响 CD34+blasts 中抗原的表达。