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Title: Hemoglobin A2 values in sickle cell disease patients quantified by high performance liquid chromatography and the influence of alpha thalassemia
Authors: Fonseca, Silvana Fahel da
Amorim, Tatiana
Purificação, Antônio
Gonçalves, Marilda
Boa Sorte, Ney
Assunto:: Hemoglobina
Talassemia
Doença falciforme
Cromatografia a líquido de alta eficiência
Issue Date: Sep-2015
Publisher: Associação Brasileira de Hematologia e Hemoterapia e Terapia Celular
Citation: FONSECA, Silvana Fahel da et al. Hemoglobin A2 values in sickle cell disease patients quantified by high performance liquid chromatography and the influence of alpha thalassemia. Revista Brasileira de Hematologia e Hemoterapia, São Paulo, v. 37, n. 5, p. 296-301, set./out. 2015. Disponível em: <http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842015000500296&lng=en&nrm=iso>. Acesso em: 2 abr. 2018. doi: http://dx.doi.org/10.1016/j.bjhh.2015.05.005.
Abstract: Background: in sickle cell disease, the quantification of Hb A2 is important for the differential diagnosis between sickle cell anemia (Hb SS) and Hb S/ß0-thalassemia. Objective: to determine Hb A2 levels as quantified by high performance liquid chromatography in patients with sickle cell anemia (Hb SS) and with the SC hemoglobinopathy, with or without concomitant alpha thalassemia. Methods: this is a retrospective study of 242 children aged between two and six years with diagnoses of Hb SS or Hb SC. The hemoglobin was evaluated using high performance liquid chromatography and alpha thalassemia [3.7 kb deletion (-a3.7)] was detected by polymerase chain reaction. Patients were classified as homozygous (-a3.7/-a3.7), heterozygous (-a3.7/a), or homozygous wild-type. Analysis of variance was used to compare the mean Hb A2 values between the alpha thalassemia groups. Results: the mean (± standard deviation) Hb A2 concentrations in the Hb SS group (n = 135) was 3.68 ± 0.65%. The mean values for individuals with Hb SS and heterozygous (n = 28) or homozygous for alpha thalassemia (n = 3) were 3.98 ± 0.64% and 4.73 ± 0.25%, respectively. The mean Hb A2 of all the Hb SC patients (n = 107) was 4.01 ± 0.507 with 4.29 ± 0.41% and 4.91 ± 0.22% in individuals heterozygous (n = 23) and homozygous for alpha thalassemia (n = 7), respectively. All patients homozygous for alpha thalassemia had Hb A2 levels above 3.5%. However, Hb A2 values above 5.2% were seen in patients with Hb SS and Hb SC, independently of alpha thalassemia. Conclusion: Hb A2 levels are elevated in patients with Hb S or Hb C, and are directly influenced by the alpha thalassemia genotypes.
Licença:: Revista Brasileira de Hematologia e Hemoterapia - This is an Open Access article distributed under the terms of the Creative Commons Attribution-Noncommercial No Derivative License, which permits unrestricted non-commercial use, distribution, and reproduction in any medium provided the original work is properly cited and the work is not changed in any way (CC BY NC ND 4.0). Fonte: http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842015000500296&lng=en&nrm=iso. Acesso em: 2 abr. 2018.
DOI: http://dx.doi.org/10.1016/j.bjhh.2015.05.005
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