부산대학교 도서관

• Recent studies have demonstrated that alterations in several immune related genes are associated with complications of SCD. In fact, higher chronic inflammatory status is related to more severe clinical symptoms in SCD patients. Although participation of innate immune cells in SCD pathogenesis has been extensively described, little is known about the roles of the adaptive immune system in this disease. Here, We reviewed the main works describing the role of immune system in SCD patients as well we emphasize that one more comprehensive understanding of immune deficiencies involved in different SCD complications is need for the development of new therapies and the improvement in the life quality and expectancy of the patient 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. [ABSTRACT FROM AUTHOR]