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Alpha thalassemia, but not β S -globin haplotypes, influence sickle cell anemia clinical outcome in a large, single-center Brazilian cohort
AbstractAlpha thalassemia and beta-globin haplotype are considered classical genetic disease modifiers in sickle cell anemia (SCA) causing clinical heterogeneity. Nevertheless, their functional impact on SCA disease emergence and progression remains elusive. To better understand the role of alpha thalassemia and beta-globin haplotype in SCA, we performed a retrospective study evaluating the clinical manifestations of 614 patients. The univariate analysis showed that the presence of alpha-thalassemia −3.7-kb mutation (αα/-α and -α/-α) decreased the risk of stroke development (p = 0.046), priapism (p = 0.033), and cho...
Source: Annals of Hematology - February 13, 2021 Category: Hematology Source Type: research

Interleukin-10 haplotypes are not associated with acute cerebral ischemia or high-risk transcranial Doppler in a newborn cohort of 395 children with sickle cell anemia
Conclusion: Interleukin-10 haplotypes were not associated with the risk of acute cerebral ischemia or high-risk transcranial Doppler velocity in children with sickle cell anemia from the state of Minas Gerais, Brazil.
Source: Revista Brasileira de Hematologia e Hemoterapia - July 3, 2017 Category: Hematology Source Type: research

High levels of proinflammatory cytokines IL-6 and IL-8 are associated with a poor clinical outcome in sickle cell anemia
AbstractSickle cell anemia (SCA) pathophysiology is characterized by the activation of sickle red blood cells, reticulocytes, leukocytes, platelets, and endothelial cells, and with the expression of several inflammatory molecules. Therefore, it is conceivable that variations in levels of proinflammatory cytokines may act as a signaling of differential clinical course in SCA. Here, we evaluated the clinical impact of proinflammatory cytokines interleukin 1- β (IL-1β), interleukin 6 (IL-6), and interleukin 8 (IL-8) in 79 patients with SCA, followed in a single reference center from northeastern Brazil. The main clinical/la...
Source: Annals of Hematology - March 4, 2020 Category: Hematology Source Type: research