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scientific articles

Role of TCD in sickle cell disease: A review

key information

source: Perspectives in Medicine

year: 2012

authors: Viviane Flumignan Zétola


Sickle cell disease (SCD) is a genetic disorder caused by homozygosity for a single β-globin gene mutation (β6GAG → GTG), in which glutamic acid has been substituted for valine at the sixth codon of the β-globin chain. Although the incidence of strokes is higher in patients with the Hb SS and Hb S/ß0 thalassemia genotype, it should be noted that strokes also occur in patients with other genotypes. The clinical course of patients suffering from SCD is extremely variable and the severity of manifestations ranging from asymptomatic to a very severe course.

SCD is characterized by chronic hemolytic anemia and intermittent vaso-occlusive events. These events result in tissue ischemia, which leads to acute and chronic pain as well as damage to any organ in the body. Acute complications include ischemic and hemorrhagic stroke, acute chest syndrome, painful vaso-occlusive crises, splenic sequestration, aplastic crises, and bacterial sepsis due to hyposplenia.

Chronic morbidities include cerebrovascular disease, pulmonary hypertension, osteonecrosis, nephropathy and organ failure. The vaso-occlusive process in SCD is of a complex nature mediated by red cell and leukocyte adhesion, inflammation, oxidative stress, and a hypercoagulable state, all resulting in endothelial injury and dysfunction.

organization: Hospital de Clínicas, Rua General Carneiro, Serviço de Neurologia, Curitiba, Brazil

DOI: 10.1016/j.permed.2012.03.016

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