Navegando por Palavras-chave "Sickle Cell Anemia"

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    Células endoteliais circulantes em diferentes tipos de tratamentos da Anemia Falciforme
    (Universidade Federal de São Paulo (UNIFESP), 2020-12-15) Souza, Claudia Regina Lustosa [UNIFESP]; Figueiredo, Maria Stella [UNIFESP]; Universidade Federal de São Paulo
    Sickle cell anemia (SCA) is characterized by anemia and pain due to chronic hemolysis and recurrent episodes of ischemia, respectively. In the presence of hypoxia, the anomalous hemoglobin (Hb), HbS, triggers microvascular occlusion, a phenomenon enhanced by the adhesion of erythrocytes and leukocytes to the vascular endothelium. As a result, vasculopathy and inflammation characteristic of SCA are observed, with the endothelial cell playing an important role. The endothelium is a dynamic, balanced tissue, where the circulation compartment reflects both the lesion and the regeneration of the vascular tree. In this compartment, circulating endothelial cells (CECs) released from vascular injury and progenitor endothelial cells (PECs) involved in vascular regeneration are identified. However, the importance of these cells in SCA and their relationship with the type of treatment still needs further clarification. Objectives: To evaluate the endothelial phenotype of individuals with sickle cell anemia and determine whether the type of treatment influences this manifestation. Casuistic: 45 patients with SCA were analyzed, followed up at the Hemoglobinopathies Outpatient Clinic of Escola Paulista de Medicina (EPM / UNIFESP). According to the treatment, the following groups were distributed: a) SS (N = 11) - steady state without hydroxyurea (HU); b) SS-HU (N = 19) – in use of maximum tolerated dose of HU; c) SS-TF (N = 15) - chronic transfusion regimen. Control group (CN, N = 13) consisting of healthy individuals. Work approved by Ethical Comittee and participants signed an informed consent form. Exclusion criteria: pregnant and puerperal women, vaso-occlusive crisis in the last 3 months, chronic renal failure and active leg ulcer. In the SS and SS-HU groups, those who received red blood cell transfusion in the last 3 months were also excluded. Methods: Laboratory evaluation: complete blood count, hemolysis tests and C-reactive protein (CRP). Identification and quantification of CECs and PECs performed by Flow Cytometry (FACSCantoII®). Results: The SS group had a higher number of CECs (median: 2.73; range: 0 - 20.0) than the CN (0.39; 0 - 4.20) and the SS-TF (0.67 ; 0 - 2.99), p = 0.016 and 0.013 respectively. The SS-HU group showed higher PECs quantification (0; 0 - 6.62) than the SS and SS-TF groups (0; 0 - 0 in both groups), p <0.05. There was no relationship between determination of CECs and clinical manifestations, however patients with priapism showed higher values (2.28; 0.74 - 6.09) than those without this manifestation (0.68; 0 - 20.0) with p = 0.055. There was no significant correlation between PECs data and clinical manifestations. Discussion & Conclusion: Our results corroborate previous studies that associate the increase in CECs with endothelial complications of SCA. The presence of fewer PECs in groups SS and SS-TF when compared to SS-HU group suggests that HU can provide endothelial protection and raises the question of its indication for all SCA patients in order to prevent serious manifestations related to endothelial dysfunction.
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    Clinical, hematological, and molecular characterization of sickle cell anemia pediatric patients from two different cities in Brazil
    (Escola Nacional de Saúde Pública Sergio Arouca, Fundação Oswaldo Cruz, 2005-08-01) Lyra, Isa Menezes; Gonçalves, Marilda Souza; Braga, Josefina Aparecida Pellegrini [UNIFESP]; Gesteira, Maria de Fátima; Carvalho, Maria Helena; Saad, Sara Teresinha Olalla; Figueiredo, Maria Stella [UNIFESP]; Costa, Fernando Ferreira; Fundação Hematologia e Hemoterapia da Bahia; Universidade Federal da Bahia; Fundação Oswaldo Cruz Centro de Pesquisa Gonçalo Moniz; Universidade Federal da Bahia Faculdade de Farmácia; Universidade Federal de São Paulo (UNIFESP); Universidade Estadual de Campinas (UNICAMP)
    This study focused on clinical, hematological, and molecular aspects of sickle cell anemia pediatric patients from two different cites in Brazil. Seventy-one patients from São Paulo and Salvador, aged 3 to 18 years, were evaluated. Hematological analyses, betaS globin gene haplotypes, and alpha2 3.7kb-thalassemia were performed. Numbers of hospitalizations due to vaso-occlusive crises, infections, stroke, and cholelithiasis were investigated. São Paulo had more hospitalizations from vaso-occlusion, cholelithiasis, and stroke than Salvador. The Ben/CAR genotype predominated in both cities. alpha2 3.7kb-thalassemia had a frequency of 28.2% in Salvador, mostly with Ben/CAR genotype (45.0%), while São Paulo had 22.5% with similar frequencies of the Ben/ CAR and CAR/CAR genotypes. Sickle cell anemia patients from São Paulo also had more episodes of stroke, which was observed among CAR/CAR, atypical, and BEN/CAR haplotypes. In Salvador stroke was only observed in the Ben/CAR genotype. Cholelithiasis had similar frequencies in the two cities. These data suggest a milder phenotype among patients in Salvador, possibly due to genetic, environmental, and socioeconomic factors. Further studies are needed to elucidate modulating factors and phenotype association.