Anemia falciforme: aspéctos genéticos e imunológicos associados à IL-8 e ao TNF-α. / Sickle cell disease: genetic and immunological aspects associated with IL-8 e ao TNF-α.

AUTOR(ES)
DATA DE PUBLICAÇÃO

2007

RESUMO

Sickle hemoglobin (HbS) presents high frequency in Brazil, and Bahia is the state with highest prevalence of sickle cell anemia. The disease has a heterogeneous clinical outcome, characterized by vaso-occlusive and infection events, contributing to a chronic proinflammatory condition. The aim of the study was investigate molecular, phenotypic and immunologic aspects in sickle cell anemia patients from Salvador-Bahia. An ambispective study was developed in a total of 126 patients, 103 in steady state and 23 hospitalized by vasoocclusive crisis and/or infection. A reference group of 212 individuals from Salvador population was also included. The α23.7Kb - thalassemia, the βS ?globin gene haplotypes, the A-251T and the G-308A gene polymorphisms located respectively in IL-8 and TNF-α genes were investigated by PCR and PCR-RFLP; the IL-8 serum levels were measured by ELISA and patients clinical history was search in clinical records. Among the steady state and crisis patients groups, infection was described in 46.6% and 78.3%, respectively. Pneumonia and crisis were more frequent in CAR/BEN genotype (p<0.05) and splenic sequestration in patients up to 5 years old (p<0.05). Pneumonia was more frequent in patients with HbF up to 10%. The α2- thalassemia 3.7Kb was found between 16.7% of patients and was associated with high HbF levels and low VCM concentration. Sickle cell anemia pediatric patients in crisis and steady state showed a high reticulocyte count and the crisis pediatric patients had a high leukocyte count (p<0,05). The IL-8 A-251T and the TNF-α G-308A gene polymorphism mutant allele were found in frequencies of 54.3% and 62% and 10.7% and 14.8% in patients and reference group, respectively. The TNF-α mutant allele was also associated with splenic sequestration (p<0.05). Interestingly, the IL-8 serum levels were 32.3+ 43.3pg/ml in patients crisis group and were still high after patients hospitalization, showing a slowly decrease. The IL-8 A allele was associated with IL-8 serun high levels, independently of crisis inductor factor. Taken together, ours results showed the role of prognostic markers interation in sickle cell anemia patients follow-up and the critical participation of the IL-8 in vaso-occlusive process. Additional studies will be necessary to establish the role as prognostic markers of IL-8 and TNF-α levels as well. as their gene polymorphisms in the sickle cell anemia clinical outcome

ASSUNTO(S)

tnf-α anemia falciforme tnfα vaso-occlusion il-8 sickle cell disease imunologia vaso-oclusão, il-8

ACESSO AO ARTIGO

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