Sickle cell disease (SCD) is caused by a single point mutation in the β-chain of the hemoglobin gene that results in the replacement of glutamic acid with valine in the hemoglobin protein. However, recent studies have demonstrated that alterations in several other genes, especially immune related genes, may be associated with complications of SCD. In fact, higher chronic inflammatory status is related to more severe clinical symptoms in SCD patients, suggesting crucial roles of the immune system in SCD physiopathology. Nevertheless, although participation of innate immune cells in SCD pathogenesis has been broadly and extensively described, little is known about the roles of the adaptive immune system in this disease. In addition, the influence of treatments on the immune system of SCD patients and their complications (such as alloimmunization) are not yet completely understood. Thus, we reviewed the current knowledge about the immune mechanisms involved in SCD pathogenesis. We suggest recommendations for future studies to allow for a broader understanding of SCD pathogenesis, helping in the development of new therapies and improvement in the life quality and expectancy of patients.

中文翻译：

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镰状细胞病发病机制中涉及的免疫机制：最新知识和观点。

镰状细胞病（SCD）是由血红蛋白基因的β链中的单点突变引起的，导致血红蛋白中的谷氨酸被缬氨酸替代。但是，最近的研究表明，其他几个基因的改变，特别是免疫相关基因的改变，可能与SCD的并发症有关。实际上，较高的慢性炎症状态与SCD患者的更严重的临床症状有关，表明免疫系统在SCD生理病理学中的关键作用。然而，尽管已经广泛和广泛地描述了先天性免疫细胞参与SCD发病机制，但关于适应性免疫系统在该疾病中的作用知之甚少。此外，治疗对SCD患者免疫系统及其并发症（如同种免疫）的影响尚未完全了解。因此，我们回顾了有关SCD发病机理的免疫机制的最新知识。我们建议对未来的研究提出建议，以使人们对SCD的发病机制有更广泛的了解，有助于开发新疗法并改善患者的生活质量和预期寿命。