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Increased red cell distribution width in Fanconi anemia: a novel marker of stress erythropoiesis

机译:范可尼贫血中红细胞分布宽度增加:应激性红细胞生成的新标志

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摘要

BackgroundRed cell distribution width (RDW), a classical parameter used in the differential diagnosis of anemia, has recently been recognized as a marker of chronic inflammation and high levels of oxidative stress (OS). Fanconi anemia (FA) is a genetic disorder associated to redox imbalance and dysfunctional response to OS. Clinically, it is characterized by progressive bone marrow failure, which remains the primary cause of morbidity and mortality. Macrocytosis and increased fetal hemoglobin, two indicators of bone marrow stress erythropoiesis, are generally the first hematological manifestations to appear in FA. However, the significance of RDW and its possible relation to stress erythropoiesis have never been explored in FA. In the present study we analyzed routine complete blood counts from 34 FA patients and evaluated RDW, correlating with the hematological parameters most consistently associated with the FA phenotype.
机译:背景技术红细胞分布宽度(RDW)是贫血鉴别诊断中的经典参数,最近被认为是慢性炎症和高水平氧化应激(OS)的标志。范可尼贫血(FA)是一种遗传疾病,与氧化还原失衡和对OS的功能障碍反应有关。在临床上,其特征在于进行性骨髓衰竭,这仍然是发病率和死亡率的主要原因。巨噬细胞增多和胎儿血红蛋白增加是骨髓应激红细胞生成的两个指标,通常是FA中最先出现的血液学表现。但是,RDW的意义及其与应激性红细胞生成的可能关系从未在FA中进行过探索。在本研究中,我们分析了34名FA患者的常规全血细胞计数,并评估了RDW,RDW与与FA表型最一致相关的血液学参数相关。

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