BIOCHEMICAL AND HEMATOLOGICAL ASPECTS OF SICKLE CELL HEMOGLOBIN

• Autor(a) principal: de Souza Botelho Machado, Laura
• Data de Publicação: 2021
• Outros Autores: Pessanha Gonçalves, Maria Júlia, Matheus da Silva, Fernanda Lorena, Haddad Kury, Charbell Miguel
• Tipo de documento: Artigo
• Idioma: por
• Título da fonte: Revista Científica da Faculdade de Medicina de Campos
• Texto Completo: https://www.fmc.br/ojs/index.php/RCFMC/article/view/499
• topic: Anemia falciforme; hemoglobina; polimerização; Sickle cell hemoglobin; hemoglobin; polymerization

Sickle cell anemia is an autosomal recessive genetic condition characterized by the alteration of the amino acid glutamic acid by valine derived from a substitution of thymine for adenine in the sixth codon in the DNA of chromosome 11. This substitution occurs in the beta chain of the hemoglobin molecule, giving rise to a anomalous molecule named hemoglobin S (HbS). The HbS formed in the absence of oxygenation has an insoluble character and, as a result, the formation of polymerized fibers occurs, resulting in the production of sickle cells. This study aims to analyze and describe the biochemical and hematological aspects of sickle cell anemia. To this end, a bibliographic review was carried out with the search for scientific articles, books and electronic media, published between 2010 and 2020. As a result of this process, the biochemical and hematological correspondence related to slowed blood flow, vascular occlusion and the appearance of organic lesions that cause inflammatory episodes, characterizing the main occurrences of the pathology.