Increased hemoglobin A2 (HbA2; ie, levels > 3.9%) is the most important feature of -thalassemia carriers. However, it is not uncommon to find persons with borderline HbA2 (levels, 3.3%-3.8%), who pose a relevant screening problem. Several genotypes have been associated with borderline HbA2, but sometimes the reasons for this unusual phenotype are unknown. In this paper, we report, for the first...

Hemoglobinopathies are the only genetic disease in which it is possible to detect carriers using hematologic findings rather than DNA analysis. Complete screening is based on the detection of red cell indices, HbA2, HbF and hemoglobin variant values. The classical phenotype of heterozygous b-thalassemia includes an elevated HbA2 level (3.4-6.0%), a relatively high red cell count, a markedly red...

Using the value of 30 mg/L for serum ferritin as cut off, 861 samples showed iron deficiency (ID) (group A) and 271 were without ID (group B) (Figure 1). The mean HbA2 value was 2.8%±0.79 in group A and 3.50%±1.23 in group B, with a significant difference (P=0.00001) among between the two groups. The distribution of the 253 samples with molecular analysis between groups A and B showed that 170 ...

Sickle cell disease (SCD) is a widespread genetic associated with severe disability and multi-organ damage, resulting in reduced life expectancy. None of the existing clinical treatments provide solution for all patients. Gene therapy fetal haemoglobin (HbF) reactivation through approaches have obtained promising, but early, results Furthermore, search active molecules to increase HbF still ong...

Introduction: Beta-thalassemia is characterized by absence or reduced synthesis of the β-globin. Carriers of β-thalassemia, typically have microcytic hypochromic anemia and elevated hemoglobin HbA2 and normal HbF level. On the other hand carriers of severe alpha-thalassemia also have similar CBC parameters to that of β-thalassemia with normal HbA2 level. Co-presence of mutations in the β-globin...

The human α-globin gene cluster is located on chromosome 16 pterp13.3, and is arranged in the order, 5’ζ2-ψζ1-ψα2-ψα1-α2-α1-θ1-3’. Recent reexamination of the ψα2 gene revealed that it is expressed at a very low level, and was renamed as mu-globin gene. The cluster was thought to result from duplication events that occurred more than 300 million years ago. Unequal homologous recombinations are ...

OBJECTIVE The capillary electrophoresis (CE) is a new system that utilizes the principle of electrokinetic separation of molecules in eight electrolyte buffer-filled silica capillaries. In this study, we established the normal ranges of haemoglobin A2 (HbA2) and haemoglobin F (HbF) levels for normal individuals using this system and also the HbA2 level in beta thalassaemia and haemoglobin E (Hb...

The Sebia Capillarys (capillary zone electrophoresis [CE]) and the Primus Resolution high-pressure liquid chromatography (HPLC) were used to prospectively evaluate 297 samples for hemoglobinopathies. Hemoglobin (Hb) A levels were similar on both techniques (mean, 96.2% and SD, 5.7% by CE; mean, 96.8% and SD, 5.5% by HPLC), but HbA2 levels were higher by CE (mean, 2.8%; SD, 0.8%) than by HPLC (m...

BACKGROUND Recent studies on the variants in duplicated human alpha globin genes (HBA2 and HBA1) actively target the α-globin gene as molecular modulators for the treatment of β-thalassemia major. Identification of the exact position of variant in HBA1, HBA2 or its patchworks is mandatory to support the therapeutic aims in β-thalassemia major, by identifying specific modulators for the reactiva...

Letter to the Editor We would like to comment on the article by Shokrani et al [ 1 ], published in the April 2000 issue of the Annals o f Clinical & Laboratory Science. The authors begin their abstract by stating that HbA2 measurements aid in the differential diagnosis of sickle cell anemia from sickle-beta-thalassemia. This, unless meant for the analysis of families, is incorrect. The HbA2 val...