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Erythroblast cell expansion as a marker for disease severity in b0-thalassemia/Hb E disease

机译:红血球细胞扩增作为b0地中海贫血/ Hb E疾病严重程度的标志

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b0-Thalassemia/Hb E disease is extremely common in many parts of Southeast Asia.? In Thailand, there are believed to be approximately 100,000 people living with this disease which can present as a life threatening anemia syndrome dependent on regular blood transfusions. Determination of the state of presentation of the disease (mild, moderate or severe) is based upon a number of clinical and hematological parameters and currently, there is no accurate predictive methodology to gauge the final severity of the disease in a particular patient. While erythropoiesis, the process generating red blood cells, takes place in the bone marrow, sufficient circulating erythroid progenitor cells are found in the peripheral blood circulation to provide materials for analysis. In this study, the expansion of isolated erythroid progenitor cells was analyzed in culture and the level of expansion assessed in relation to disease severity. A clear statistically significant relationship between erythroid progenitor expansion and disease severity was observed, providing for the first time a potential predictive marker for disease severity for?b0-thalassemia/Hb E disease.
机译:在东南亚许多地区,b0-地中海贫血/ Hb E疾病极为普遍。在泰国,据信大约有100,000人患有这种疾病,可表现为依赖定期输血的威胁生命的贫血综合症。疾病表现状态(轻度,中度或重度)的确定基于许多临床和血液学参数,目前,尚无准确的预测方法来评估特定患者的疾病最终严重程度。在骨髓中发生红细胞生成(红细胞生成过程)时,在外周血循环中发现了足够的循环红系祖细胞,以提供分析材料。在这项研究中,在培养中分析了分离的类红细胞祖细胞的扩增,并评估了与疾病严重性相关的扩增水平。观察到红细胞祖细胞扩增与疾病严重程度之间的统计学显着相关性,这首次为潜在的预测性标志物提供了针对bb0地中海贫血/ Hb E疾病的严重程度。

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