| Autor: |
da Silva Araújo A; Department of Hematology, Fundação de Hematologia e Hemoterapia de Pernambuco, Recife, Brazil., Silva Pinto AC; Department of Medical Imaging, Hematology and Oncology, Hemocentro de Ribeirão Preto, Hospital das Clínicas da Faculdade de Medicina de Ribeirão Preto - USP, Ribeirão Preto, Brazil., de Castro Lobo CL; Department of Clinical Research, Instituto Estadual de Hematologia Arthur de Siqueira Cavalcanti (HEMORIO), Rio de Janeiro, RJ, Brazil., Figueiredo MS; Department of Clinical and Experimental Oncology, Escola Paulista de Medicina da Universidade Federal de São Paulo (UNIFESP), São Paulo, Brazil., Menosi Gualandro SF; Department of Hematology, Hospital das Clínicas da Faculdade de Medicina da USP, São Paulo, Brazil., Olalla Saad ST; Department of Internal Medicine, Faculdade de Ciências Médicas - Unicamp, Campinas, Brazil., Cançado RD; Department of Hematology and Oncology, Faculdade de Ciências Médicas da Santa Casa de São Paulo, São Paulo, Brazil. |
| Abstrakt: |
Sickle cell disease (SCD) comprises inherited red blood cell disorders due to a mutation in the β-globin gene (c20A > T, pGlu6Val) and is characterized by the presence of abnormal hemoglobin, hemoglobin S, hemolysis, and vaso-occlusion. This mutation, either in a homozygous configuration or in compound states with other β-globin mutations, leads to polymerization of hemoglobin S in deoxygenated conditions, causing modifications in red blood cell shape, particularly sickling. Vaso-occlusive crisis (VOC) is the hallmark of the disease, but other severe complications may arise from repeated bouts of VOCs. SCD is considered a global health problem, and its incidence has increased in some areas of the world, particularly the Americas and Africa. Management of the disease varies according to the region of the world, mainly due to local resources and socioeconomic status. This review aimed to describe more recent data on SCD regarding available treatment options, especially in Brazil. New treatment options are expected to be available to all patients, particularly crizanlizumab, which is already approved in the country. |
