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首页> 外文期刊>Advances in enzyme regulation >Characterization of the hemopoietic defect in early stages of the myelodysplastic syndromes.
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Characterization of the hemopoietic defect in early stages of the myelodysplastic syndromes.

机译：骨髓增生异常综合症早期阶段造血缺陷的特征。

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Myelodysplasia (MDS) is a heterogeneous disorder characterised by bone marrow failure and progression to acute myeloid leukaemia where the molecular and cellular haematopoietic defects are poorly understood. To gain insight into this the pathogenesis of this condition, we analyzed gene expression profiles of bone marrow CD34+ stem/progenitor cells from patients with MDS at a early stage in the disease and compared them with profiles from CD34+ cells from age-matched controls and patients with non-MDS anaemia. Given the heterogeneity of early MDS, a surprisingly consistent finding was decreased expression of B-cell lineage affiliated genes in MDS patients compared to normal controls and samples with non-MDS anaemia. These findings were then confirmed in the original samples and further samples from a new MDS patient group by Taqman Real Time PCR. Flow cytometry on unfractionated marrow from independent samples also demonstrated reduced B-cell progenitors in MDS patients compared to normal controls. These novel findings suggest a common perturbation in early MDS haematopoiesis. They also provide the rationale for a larger study to evaluate the diagnostic utility of reduced B-cell progenitor number as a diagnostic biomarker of early low risk MDS which can pose a diagnostic challenge.
机译：骨髓增生异常（MDS）是一种异质性疾病，其特征是骨髓衰竭并发展为急性髓细胞性白血病，其中对分子和细胞的造血功能缺陷了解甚少。为了深入了解这种疾病的发病机理，我们分析了疾病早期阶段MDS患者骨髓CD34 +干/祖细胞的基因表达谱，并将其与年龄匹配的对照组和患者的CD34 +细胞谱进行比较非MDS贫血。鉴于早期MDS的异质性，与正常对照和非MDS贫血样本相比，MDS患者中B细胞谱系相关基因的表达降低是一个令人惊讶的一致发现。然后，通过Taqman Real Time PCR在原始样品和新的MDS患者组的其他样品中证实了这些发现。与正常对照组相比，对来自独立样本的未分离的骨髓进行流式细胞术也证明了MDS患者的B细胞祖细胞减少。这些新颖的发现提示早期MDS造血过程中常见的扰动。他们还为进行更大规模研究以评估降低的B细胞祖细胞数量作为早期低风险MDS的诊断生物标记物的诊断依据提供了理论依据。

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