Avaliação do perfil hematológico de portadores de talassemia alfa provenientes das regiões Sudeste e Nordeste do Brasil
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Data
2006-06-01
Autores
Oliveira, Gislane L. V. [UNESP]
Mendiburu, Carlos F. [UNESP]
Bonini-Domingos, Claudia R. [UNESP]
Título da Revista
ISSN da Revista
Título de Volume
Editor
Associação Brasileira de Hematologia e Hemoterapia e daSociedade Brasileira de Transplante de Medula Óssea
Resumo
A talassemia alfa é uma anemia hereditária resultante da síntese deficiente de cadeias alfa, provocando um excesso relativo de cadeias beta, que vão formar tetrâmeros identificados como hemoglobina H (Hb H) no indivíduo adulto. Para direcionar o diagnóstico laboratorial desta anemia, a análise dos índices eritrocitários, a eletroforese em acetato de celulose em pH neutro e a pesquisa de corpos de inclusão de Hb H são essenciais. O objetivo deste estudo foi traçar o perfil hematológico, por meio dos índices eritrocitários, dos portadores de talassemia alfa das regiões Sudeste e Nordeste do Brasil. Foram analisadas 1.010 amostras de sangue periférico após consentimento informado. Os índices eritrocitários como contagem de glóbulos vermelhos (RBC), dosagem de hemoglobina (HGB), hematócrito (HCT), volume corpuscular médio (VCM), hemoglobina corpuscular média (HCM) e concentração de hemoglobina corpuscular média (CHCM) foram fornecidos por aparelhos automatizados com controle de qualidade interno e externo. Para o diagnóstico de talassemia alfa foram utilizados testes de triagem e complementares para talassemias, como eletroforese em pH neutro e pesquisa de corpos de inclusão de Hb H com coloração de azul cresil brilhante. Comparando-se os valores hematológicos observados nos dois grupos, notou-se que, em ambas as regiões, os índices com valores discrepantes foram os níveis de HGB e HCT, sendo a maior freqüência de variação observada entre as mulheres. Nos portadores do fenótipo alfa talassêmico da região Nordeste, todos os índices eritrocitários estavam abaixo dos valores de normalidade. Estes resultados evidenciam a necessidade de melhor avaliação do perfil hematológico de talassemia alfa em diferentes regiões, considerando-se os interferentes ambientais para um diagnóstico mais preciso.
Alpha thalassemia is a hereditary anaemia resulting from deficient synthesis of the alpha chains, causing a relative excess of beta chains which form tetramers identified as hemoglobin H (Hb H) in adults. To diagnose this type of anaemia it is essential to analyze the erythrocytic values, electrophoresis in neutral pH and to study the Hb H inclusion bodies. The aim of this study was to determine the hematologic profile of alpha thalassemia carriers from the southeastern and northeastern regions of Brazil. Peripheral blood samples from 1010 patients were analyzed. The erythrogram values, including the red blood cell count (RBC), hemoglobin level (HGB), hematocrit (HCT), mean cell volume (MCV), mean cell hemoglobin (MCH) and mean cell hemoglobin concentration (MCHC) were supplied by automatic devices. To diagnose alpha thalassemia, screening and complementary tests were performed, including electrophoresis in neutral pH and a study of the Hb H inclusion bodies with brilliant blue cresil staining. Comparing hematologic values obtained in both groups, the indices with discrepant values were related to HGB and HCT and the greatest variations in both regions were observed among women. In alpha thalassemic phenotypic carriers from the northeastern region, all the erythrocytic values were lower than normal. These results show the necessity of better evaluation of alpha thalassemia in different regions, considering the environmental factors in order to give an adequate diagnosis.
Alpha thalassemia is a hereditary anaemia resulting from deficient synthesis of the alpha chains, causing a relative excess of beta chains which form tetramers identified as hemoglobin H (Hb H) in adults. To diagnose this type of anaemia it is essential to analyze the erythrocytic values, electrophoresis in neutral pH and to study the Hb H inclusion bodies. The aim of this study was to determine the hematologic profile of alpha thalassemia carriers from the southeastern and northeastern regions of Brazil. Peripheral blood samples from 1010 patients were analyzed. The erythrogram values, including the red blood cell count (RBC), hemoglobin level (HGB), hematocrit (HCT), mean cell volume (MCV), mean cell hemoglobin (MCH) and mean cell hemoglobin concentration (MCHC) were supplied by automatic devices. To diagnose alpha thalassemia, screening and complementary tests were performed, including electrophoresis in neutral pH and a study of the Hb H inclusion bodies with brilliant blue cresil staining. Comparing hematologic values obtained in both groups, the indices with discrepant values were related to HGB and HCT and the greatest variations in both regions were observed among women. In alpha thalassemic phenotypic carriers from the northeastern region, all the erythrocytic values were lower than normal. These results show the necessity of better evaluation of alpha thalassemia in different regions, considering the environmental factors in order to give an adequate diagnosis.
Descrição
Palavras-chave
Anaemias, alpha thalassemia, red blood cells, Anemias, Talassemia alfa, Índices eritrocitários
Como citar
Revista Brasileira de Hematologia e Hemoterapia. Associação Brasileira de Hematologia e Hemoterapia e daSociedade Brasileira de Transplante de Medula Óssea, v. 28, n. 2, p. 105-109, 2006.