Centro de Investigación del Cáncer (Instituto de Biología Molecular y Celular del Cáncer; CSIC-USAL), Servicio General de Citometría and Departamento de Medicina, Universidad de Salamanca, Salamanca, Spain.
Cytometry B Clin Cytom. 2010 May;78(3):154-68. doi: 10.1002/cyto.b.20513.
A heterogeneous spectrum of immunophenotypic abnormalities have been reported in myelodysplastic syndromes (MDS). However, most studies are restricted to the analysis of CD34(+) cells and/or other major subsets of CD34(-) cells, frequently not exploring the diagnostic and prognostic impact of immunophenotyping.
We propose for the first time an immunophenotypic score (IS) based on the altered distribution and immunophenotypic features of maturing/mature compartments of bone marrow (BM) hematopoietic cells in 56 patients with MDS that could contribute to a refined diagnosis and prognostic evaluation of the disease.
Although MDS-associated phenotypes were detected in reactive BM, the overall immunophenotypic profile of BM cells allowed an efficient discrimination between MDS and both normal and reactive BM, once the number and degree of severity of the abnormalities detected per patient were simultaneously considered in the proposed IS. Interestingly, increasingly higher IS were found among patients with MDS showing adverse prognostic factors and in low- versus high-grade cases. The most informative prognostic factors included the number of CD34(+) cells, presence of aberrant CD34(-)/CD117(+) precursors, decreased mature neutrophils and CD34(-) erythroid precursors, and increased numbers of CD36(-/lo) erythroid precursors; in addition, the IS was an independent prognostic factor for overall survival.
Assessment of immunophenotypic abnormalities of maturing/mature BM cells allows an efficient discrimination between MDS and both normal and reactive BM, once the number and degree of severity of the abnormalities detected are simultaneously scored. Interestingly, progressively higher IS were found among patients with MDS with adverse prognostic features and shorter overall survival.
骨髓增生异常综合征（MDS）的免疫表型异常具有异质性。然而，大多数研究仅限于 CD34(+)细胞和/或其他 CD34(-)细胞的主要亚群分析，通常不探讨免疫表型的诊断和预后影响。
我们首次提出了一种基于骨髓（BM）造血细胞成熟/成熟区室中改变的分布和免疫表型特征的免疫表型评分（IS），该评分可有助于对疾病进行更精细的诊断和预后评估。
尽管在反应性 BM 中检测到 MDS 相关表型，但 BM 细胞的整体免疫表型谱允许有效区分 MDS 与正常和反应性 BM，一旦同时考虑每个患者检测到的异常的数量和严重程度。有趣的是，在具有不良预后因素和低级别与高级别病例中，发现越来越高的 IS。最具信息性的预后因素包括 CD34(+)细胞数量、存在异常 CD34(-)/CD117(+)前体、成熟中性粒细胞和 CD34(-)红细胞前体减少，以及 CD36(-/lo)红细胞前体数量增加；此外，IS 是总生存期的独立预后因素。
评估成熟/成熟 BM 细胞的免疫表型异常可有效区分 MDS 与正常和反应性 BM，一旦同时评分检测到的异常的数量和严重程度。有趣的是，在具有不良预后特征和总生存期较短的 MDS 患者中，发现 IS 逐渐升高。
