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Immunophenotyping in Myelodysplastic Syndromes Can Add Prognostic Information to Well-Established and New Clinical Scores

机译:骨髓增生异常综合症的免疫分型可以为成熟的和新的临床评分增加预后信息

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摘要

Background: myelodysplastic syndromes (MDS) are a heterogeneous group of hematopoietic clonal disorders. So, prognostic variables are important to separate patients with a similar biology and clinical outcome. We compared the importance of risk stratification in primary MDS of IPSS and WPSS with the just described revision of IPSS (IPSS-R), and examined if variables obtained by bone marrow immunophenotyping could add prognostic information to any of the scores. Methods: In this prospective study of 101 cases of primary MDS we compared the relation of patients' overall survival with WHO types, IPSS, IPSS-R, WPSS and phenotypic abnormalities of hematopoietic precursors. We examined aberrancies in myelomonocytic precursors and CD34(+) cells. Patients were censored when receiving chemotherapy or BM transplantation. Survival analysis was made by Cox regressions and stability of the models was examined by bootstrap resampling. Results: median age: 64 years (15-93). WHO types: 2 cases of 5q-syndrome, 7 of RA, 64 of RCDM and 28 of RAEB. In the univariate Cox analysis, increasing risk category of all scores, degree of anemia, higher percentage of BM blasts, higher number of CD34(+) cells and their myeloid fractions besides increasing number of phenotypic abnormalities detected were significantly associated with a shorter survival. In the multivariate analysis comparing the three scores, IPSS-R was the only independent risk factor. Comparing WPSS with phenotypic variables (CD34(+)/CD13(+) cells, CD34(+)/CD13(-) cells and 'total alterations') the score and 'CD34(+)/CD13(+) cells' remained in the model. When IPSS was tested together with these phenotypic variables, only 'CD34(+)/CD13(+) cells', and 'total alterations' remained in the model. Testing IPSS-R with the phenotypic variables studied, only the score and 'CD34(+)/CD13(+) cells' entered the model. Conclusions: Immunophenotypic analysis of myelomonocytic progenitors provides additional prognostic information to all clinical scores studied. IPSS-R improved risk stratification in MDS compared to the former scores.
机译:背景:骨髓增生异常综合症(MDS)是造血克隆疾病的异质性群体。因此,预后变量对于分离生物学和临床结局相似的患者很重要。我们将IPSS和WPSS的主要MDS与刚刚描述的IPSS修订版(IPSS-R)进行风险分层的重要性进行了比较,并研究了通过骨髓免疫表型获得的变量是否可以为任何评分增加预后信息。方法:在这项对101例原发性MDS患者的前瞻性研究中,我们比较了患者总体生存率与WHO类型,IPSS,IPSS-R,WPSS和造血前体表型异常之间的关系。我们检查了骨髓单核细胞前体和CD34(+)细胞中的畸变。接受化学疗法或BM移植时对患者进行检查。通过Cox回归进行生存分析,并通过bootstrap重采样检查模型的稳定性。结果:中位年龄:64岁(15-93)。世卫组织类型:2例5q综合征,7例RA,64例RCDM和28例RAEB。在单变量Cox分析中,除了检测到的表型异常数目增加之外,所有评分,贫血程度,BM blast百分比更高,CD34(+)细胞数目更高以及它们的髓样分数的风险类别增加与存活时间短显着相关。在比较这三个评分的多元分析中,IPSS-R是唯一的独立危险因素。将WPSS与表型变量(CD34(+)/ CD13(+)细胞,CD34(+)/ CD13(-)细胞和“总改变”)进行比较,得分和“ CD34(+)/ CD13(+)细胞”仍然存在该模型。当将IPSS与这些表型变量一起测试时,模型中仅保留“ CD34(+)/ CD13(+)细胞”和“总改变”。用研究的表型变量测试IPSS-R,只有得分和“ CD34(+)/ CD13(+)细胞”才进入模型。结论:骨髓单核祖细胞的免疫表型分析为所有研究的临床评分提供了更多的预后信息。与以前的评分相比,IPSS-R改善了MDS中的风险分层。

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