Li Jin, He Guang-Sheng
Key Laboratory of Thrombosis and Hemostasis, Ministry of Health, Jiangsu Proviucial Institute of Hematology, The First Affiliated Hospital of Soochow University,Suzhou 215006, Jiangsu Province,China.
Zhongguo Shi Yan Xue Ye Xue Za Zhi. 2013 Aug;21(4):1069-72. doi: 10.7534/j.issn.1009-2137.2013.04.049.
Myelodysplastic syndrome (MDS) is a heterogeneous disease characterized by dysplasia and ineffective hematopoiesis. The dysplasia is crucial in the diagnosis of MDS, but the morphologic abnormalities of bone marrow cells are not specific for MDS. When the morphological evaluation of marrow dysplasia and cytogenetics can not give enough informations, for diagnosis of MDS, the application of flow cytometry (FCM) for immunophenotyping in MDS will become particularly important. Multiparametric evaluation of myeloid, monocytic maturation and antigen expression pattern contribute to the identification of two or more aberrancies in MDS cases. FCM evaluation of erythroid dysplasia is particularly difficult, because of the limited availability of specific markers. By analyzing the proteins involved in cellular iron metabolism, MDS erythroid cells present an "iron-loaded" phenotype characterized by increased ferritin contents and reduced transferrin receptor, which reflects the degree of dysplasia assessed by morphology. The proportion of CD34(+) cells increased, abnormal expression of surface antigen is also important. The application of flow cytometry in detecting dysplasia of myelodysplastic syndrome is discussed in this article.
骨髓增生异常综合征(MDS)是一种以发育异常和无效造血为特征的异质性疾病。发育异常在MDS的诊断中至关重要,但骨髓细胞的形态学异常并非MDS所特有。当骨髓发育异常的形态学评估和细胞遗传学检查不能提供足够信息时,对于MDS的诊断,应用流式细胞术(FCM)进行MDS免疫表型分析就显得尤为重要。对髓系、单核细胞成熟及抗原表达模式进行多参数评估有助于识别MDS病例中的两种或更多异常情况。由于特异性标志物有限,FCM评估红系发育异常尤其困难。通过分析参与细胞铁代谢的蛋白质,MDS红系细胞呈现出一种“铁负荷”表型,其特征为铁蛋白含量增加和转铁蛋白受体减少,这反映了通过形态学评估的发育异常程度。CD34(+)细胞比例增加、表面抗原异常表达也很重要。本文探讨了流式细胞术在检测骨髓增生异常综合征发育异常中的应用。