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Phenotypical and functional abnormalities of circulating neutrophils in patients with β-thalassemia.

Β-地中海贫血患者循环中性粒细胞的表型和功能异常。

  • 影响因子:1.94
  • DOI:10.1007/s00277-020-04213-0
  • 作者列表:"Buttari B","Profumo E","Caprari P","Massimi S","Sorrentino F","Maffei L","Gabbianelli M","Riganò R
  • 发表时间:2020-10-01
Abstract

:β-Thalassemia is an inherited single gene disorder related to reduced synthesis of the β-globin chain of hemoglobin. Patients with β-thalassemia present variable clinical severity ranging from asymptomatic trait to severe transfusion-dependent anemia and multiple organs complications. Moreover, multiple immune abnormalities are a major concern in β-thalassemia patients. Aberrant neutrophil effector function plays a pivotal role in infection susceptibility in these patients. In severe and persistent inflammation, immature neutrophils are released from the bone marrow and are functionally different compared with mature ones. Despite some abnormalities reported for thalassemia patient's immune system, few data exist on the characterization of human neutrophils in β-thalassemia. The aim of this study was to investigate the phenotype and function of circulating neutrophil subsets in patients with β-thalassemia major and with β-thalassemia intermedia divided in transfusion-dependent and non-transfusion-dependent. By the use of immunochemical and cytofluorimetric analyses, we observed that patients' CD16+ neutrophils exhibit abnormalities in their phenotype and functions and the abnormalities vary according to the clinical form of the disease and to the neutrophil subset (CD16bright and CD16dim). Abnormalities include altered surface expression of the innate immune receptor CD45, Toll-like receptor 4, and CD32, reduced ability to produce an oxidative burst, and elevated levels of membrane lipid peroxidation, especially in patients with a more severe form of the disease. Overall, our results indicating the occurrence of an immuno-senescent phenotype on circulating neutrophils from thalassemia patients suggest the usefulness of neutrophil feature assessment as a tool for better clinical management of β-thalassemia.

摘要

: β-地中海贫血是一种遗传性单基因疾病,与血红蛋白 β-珠蛋白链合成减少有关。Β-地中海贫血患者呈现从无症状特征到严重输血依赖性贫血和多器官并发症的可变临床严重程度。此外,多重免疫异常是 β-地中海贫血患者的主要关注点。异常的中性粒细胞效应子功能在这些患者的感染易感性中起关键作用。在严重和持续的炎症中,未成熟的中性粒细胞从骨髓中释放出来,并且与成熟的中性粒细胞相比在功能上是不同的。尽管报道了地中海贫血患者免疫系统的一些异常,但关于 β-地中海贫血中的人中性粒细胞的特征的数据很少。本研究的目的是调查重型 β-地中海贫血和中间 β-地中海贫血患者循环中性粒细胞亚群的表型和功能,分为输血依赖性和非输血依赖性。通过使用免疫化学和细胞荧光分析,我们观察到患者的CD16 + 嗜中性粒细胞在其表型和功能方面表现出异常,并且异常根据疾病的临床形式和嗜中性粒细胞亚群 (CD16bright和CD16dim) 而变化。异常包括先天免疫受体CD45、Toll样受体4和CD32的表面表达改变,产生氧化爆发的能力降低,以及膜脂质过氧化水平升高,特别是在具有更严重形式的疾病的患者中。总的来说,我们的结果表明地中海贫血患者循环中性粒细胞出现免疫衰老表型,这表明中性粒细胞特征评估作为更好地治疗 β-地中海贫血的工具是有用的。

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影响因子:1.74
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翻译标题与摘要 下载文献
血红蛋白病方向

由于血红蛋白分子结构异常(异常血红蛋白病),或珠蛋白肽链合成速率异常(珠蛋白生成障碍性贫血,又称海洋性贫血)所引起的一组遗传性血液病。

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