ABSTRACT
Accurate and precise hemoglobin separation and the quantitation of Hb A2 and Hb F are essential for the diagnosis of the thalassemias and hemoglobinopathies. Presented in this study is the validation of the the Hb A2 assay of the HbGold analyzer, a fully automated liquid chromatography system for hemoglobin separation and quantitation. Variability of Hb A2 quantitation was quite low; the CV's of within-run, between-run and interlaboratory studies were 1.8-3.1%, 3.4-6.0% and 6.8-8.8% respectively. The results of the %Hb A2 quantitated by HbGold analyzer correlated well with those given by the Bio-Rad Variant Hb testing system (r=0.98). The application of the HbGold analyzer for the diagnosis of the thalassemia phenotypes frequently observed in Thailand is considered. In conclusion, the Hb A2 assay of the HbGold analyzer could be used for the quantitation of Hb A2 and Hb F and the presumptive identification of abnormal hemoglobins.
Subject(s)
Automation/methods , Bias , Case-Control Studies , Chromatography, Liquid/instrumentation , Fetal Hemoglobin/metabolism , Hemoglobin A2/metabolism , Hemoglobin E/metabolism , Hemoglobinopathies/blood , Genetic Carrier Screening/methods , Homozygote , Humans , Immunoblotting , Linear Models , Phenotype , Polymerase Chain Reaction , Sensitivity and Specificity , Thailand/epidemiology , Thalassemia/bloodABSTRACT
AIM: To study the activity of superoxide dismutase (SOD) and the level of fetal hemoglobin (HbF) in hydroxyurea (HU)-treated E beta thalassaemia. METHODS: We have measured SOD level, HbF, mean corpuscular volume (MCV), packed cell volume (PCV) and hemoglobin (Hb) of E beta thalassaemia treated with HU (dose 30 mg/kg/day) for consecutive 90 days. RESULT: The increase of HbF synthesis without increase of Hb was observed in HU-treated patients. CONCLUSION: The decreased SOD level in long term and low dose of HU therapy in E beta thalassaemia may have some role to inhibit superoxide radical of erythrocyte. HU may act as inhibitor for oxidative damage of red cell in E beta thalassaemia.
Subject(s)
Adolescent , Antisickling Agents/therapeutic use , Fetal Hemoglobin/metabolism , Humans , Hydroxyurea/therapeutic use , Superoxide Dismutase/metabolism , beta-Thalassemia/drug therapyABSTRACT
O presente trabalho se refere a importancia da hiperglicemia na síntese de hemoglobina F. Foram estudados 1027 indivíduos normais e 226 com hiperglicemia (diabéticos descompensados). A hemoglobina F foi dosada pela técnica da Desnaturaçäo alcalina de Betke e cols (1969). Os nossos resultados mostraram que: 1) nos indivíduos com hiperglicemia os níveis de hemoglobina F estäo diminuidos quando comparados com os níveis apresentados pelos indivíduos normais; 2) os níveis diminuídos de hemoglobina F näo estiveram relacionados com determinadas condiçöes que estäo presentes nos diabéticos - faixa etária, tipo de diabete I e II, período da diabete no indivíduo (meses, anos) e evoluçäo clínica da diabete (presença ou ausência de coma). Discute-se sobre a açäo da eritropoietina e da hiperglicemia na eritropoiese: a) acredita-se que a eritropoietina e a hiperglicemia atuam de modo diferente na celula precursora eritróide (BFU-E); b) a eritropoietina estimulando as divisöes celulares tendo como consequencia maior ou menor número de hemácias circulantes; c) a hiperglicemia interferindo na diminuiçäo da produçäo de hemoglobina F. Conclue-se admitindo-se que, o padräo de formaçäo de hemoglobina F no tecido adulto hematopoiético pode ser reversivel na dependência do ambiente metabólico celular, e que, a elucidaçäo destes mecanismos pode ser de particular importância para ajudar no diagnóstico e ou tratamento de variadas disordens hereditárias e adquiridas que envolvem alteraçöes da hemoglobina F