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The intriguing contribution of white blood cells to sickle cell disease - a red cell disorder.
Blood Reviews ( IF 6.9 ) Pub Date : 2003-12-20 , DOI: 10.1016/s0268-960x(03)00037-7
Iheanyi Okpala 1
Affiliation  

Sickle cell disease (SCD) is characterized by a point mutation that replaces adenine with thymidine in the sixth codon of the beta-globin gene, a unique morphological abnormality of red blood cells, vaso-occlusion with ischaemic tissue injury, and susceptibility to infections. Vascular lumen obstruction in SCD results from interaction of erythrocytes, leukocytes, platelets, plasma proteins, and the vessel wall. The disease phenotype is a product of various genes and environmental factors acting in concert with the protein lesion underlying the red cell anomaly. The severity of SCD increases with leukocyte count. The biological basis and therapeutic implications of this relationship are discussed. Leukocytes contribute to SCD by adhering to blood vessel walls and obstructing the lumen, aggregating with other blood cells with more effective blockage of the lumen, stimulating the vascular endothelium to increase its expression of ligands for adhesion molecules on blood cells, and causing tissue damage and inflammatory reaction which predispose to vaso-occlusion. Patients with impaired ability of leukocytes to kill microbes are more prone to infections; which precipitate sickle cell crisis. Reduction of leukocyte count ameliorates SCD. Similarly, targeted blockade or reduced synthesis of specific leukocyte adhesion molecules and their ligands might confer clinical benefit in SCD.

中文翻译:

白细胞对镰状细胞病-红细胞疾病的有趣贡献。

镰状细胞病(SCD)的特征在于,β-珠蛋白基因第六个密码子中的点突变被胸腺嘧啶替换为腺嘌呤,红细胞的独特形态异常,缺血性组织损伤引起的血管闭塞以及对感染的易感性。SCD中的血管腔阻塞是由红细胞,白细胞,血小板,血浆蛋白和血管壁的相互作用引起的。疾病表型是各种基因和环境因素与红细胞异常背后的蛋白质病变协同作用的产物。SCD的严重程度随白细胞计数增加。讨论了这种关系的生物学基础和治疗意义。白细胞通过粘附在血管壁上并阻塞管腔来促进SCD,与其他血细胞聚集在一起,更有效地阻塞管腔,刺激血管内皮以增加其在血细胞上粘附分子的配体的表达,并引起组织损伤和炎症反应,从而易于阻塞血管。白细胞杀死微生物能力受损的患者更容易感染。从而引发镰状细胞危机。减少白细胞计数可改善SCD。同样,特异性阻断或减少特定白细胞粘附分子及其配体的合成可能会赋予SCD临床益处。白细胞杀死微生物能力受损的患者更容易感染。从而引发镰状细胞危机。减少白细胞计数可改善SCD。同样,特异性阻断或减少特定白细胞粘附分子及其配体的合成可能会赋予SCD临床益处。白细胞杀死微生物能力受损的患者更容易感染。从而引发镰状细胞危机。减少白细胞计数可改善SCD。同样,特异性阻断或减少特定白细胞粘附分子及其配体的合成可能会赋予SCD临床益处。
更新日期:2019-11-01
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