Abstract | BACKGROUND: OBJECTIVE: 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 [3.7kb deletion (-α(3.7))] was detected by polymerase chain reaction. Patients were classified as homozygous (-α(3.7)/-α(3.7)), heterozygous (-α(3.7)/α), 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.
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Authors | Silvana Fahel da Fonseca, Tatiana Amorim, Antônio Purificação, Marilda Gonçalves, Ney Boa-Sorte |
Journal | Revista brasileira de hematologia e hemoterapia
(Rev Bras Hematol Hemoter)
2015 Sep-Oct
Vol. 37
Issue 5
Pg. 296-301
ISSN: 1516-8484 [Print] Brazil |
PMID | 26408362
(Publication Type: Journal Article)
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Copyright | Copyright © 2015 Associação Brasileira de Hematologia, Hemoterapia e Terapia Celular. Published by Elsevier Editora Ltda. All rights reserved. |