Abstract
The Impact of Repeated Hb A2 Measurements on β-Thalassemia Trait Diagnosis
Highlights
Hemoglobin A2 (Hb A2) is a minor component of the hemoglobin present in normal adult red blood cells, accounting for 1.5-3.5% of the total hemoglobin in healthy individuals
Alert technical work and careful clinical interpretation are mandatory to avoid wrong diagnosis related to Hb A2 level in β-thalassemia trait and other related hemoglobin disorders
Analysis of the results of the 107 repeat tested samples revealed that 51 (47%) samples were in the 0.25% variant interval ranges (VIRs); 28 (26%) samples were in the 0.5% VIR; 14 (13%) samples were in the 0.75% VIR; 5 (5%) samples were in the 1.00% VIR; 2 (2%) samples were in the 1.25% VIR; and 7 (7%) samples were in the 1.5% VIR
Summary
Hemoglobin A2 (Hb A2) is a minor component of the hemoglobin present in normal adult red blood cells, accounting for 1.5-3.5% of the total hemoglobin in healthy individuals. Hemoglobin A2 (Hb A2) level is the most significant parameter in the identification of β-thalassemia carriers. Reduced Hb A2 levels can be detected in the presence of α-thalassemia mutations, probably due to preferential binding of the α-chains with the β rather than with their δ counterparts. This is clearly seen in hemoglobin H disease (Hb H), where Hb A2 can drop to less than 1% [7]. The Hb A2 variations detected by repeated HPLC tests in this study could potentially influence the diagnosis of the β-thalassemia trait and another hemoglobinopathies. This study highlights the impact of such variations on Hb A2 level quality control determined by HPLC testing
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