Immune mechanisms involved in sickle cell disease pathogenesis: current knowledge and perspectives

Author(s)

Cottas de Azevedo, Julia Teixeira ^{[1, 2]} ; Ribeiro Malmegrim, Kelen Cristina ^{[3, 2]}

Affiliation

^[1] Univ Sao Paulo, Ribeirao Preto Med Sch, Dept Biochem & Immunol, Ribeirao Preto - Brazil

^[2] Univ Sao Paulo, Ctr Cell Based Therapy, Reg Blood Ctr Ribeirao Preto, Ribeirao Preto Med Sch, Ribeirao Preto - Brazil

^[3] Univ Sao Paulo, Sch Pharmaceut Sci Ribeirao Preto, Dept Clin Toxicol & Bromatol Anal, Ave Cafe S-N, BR-14010903 Ribeirao Preto, SP - Brazil

• Document type: Review article
• Source: Immunology Letters; v. 224, p. 1-11, AUG 2020.
• Web of Science Citations: 0

Abstract

Sickle cell disease (SCD) is caused by a single point mutation in the beta-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. (AU)

• FAPESP's process: 13/08135-2 - CTC - Center for Cell-Based Therapy
• Grantee: Dimas Tadeu Covas
• Support Opportunities: Research Grants - Research, Innovation and Dissemination Centers - RIDC
• FAPESP's process: 14/00088-8 - Haematological and immunological mechanisms involved in the therapeutic response of patients with sickle cell anemia submitted to allogeneic hematopoietic stem cell transplantation
• Grantee: Júlia Teixeira Cottas de Azevedo
• Support Opportunities: Scholarships in Brazil - Doctorate