ICSH recommendations for the measurement of Haemoglobin A<sub>2</sub>

Stephens, A. D.; Angastiniotis, M.; Baysal, E.; Chan, Vny; Fucharoen, Suthat; Giordano, PC; Hoyer, JD; Mosca, Andrea; Wild, Barbara

doi:10.1111/j.1751-553x.2011.01368.x

Cited by 97 publications

(76 citation statements)

References 37 publications

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“…As previously reported, values below 2.5 or 2% are however measured in homozygous a+ (-a/-a) or heterozygous a° (-/aa) conditions, reaching levels around 1.5% in HbH disease (-/-a) 15 (summarized in Table 1). …”

Section: Hemoglobin a 2 In A-thalassemia Conditionssupporting

confidence: 67%

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HbA2 Measurements in β-Thalassemia and in Other Conditions

Ivaldi

Barberio

Harteveld

et al. 2014

Thalassemia Reports

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Quite a few papers have been written on the significance of elevated hemoglobin (Hb) A 2 as a parameter for the diagnosis of b-thalassemia trait, on the cutoff values to be used in diagnostics and on the significance and effects of factors reducing or elevating the expression of HbA 2 and last but not least on the need for reliable measurement methods and precise calibrations with accurate standards. However, little has been published on the causes that elevate or reduce the HbA 2 levels in b-and a-thalassemia and in other conditions. For a better understanding of the value of a precise measurement of this parameter we summarize and elucidate in this review the direct and indirect mechanisms that cause the variations in HbA 2 expression and that influence the value of this parameter in particular conditions. We conclude by explaining the advantages and disadvantages of trusting on a precise measurement in the complete diagnostic contest.

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Section: Hemoglobin a 2 In A-thalassemia Conditionssupporting

confidence: 67%

“…Although apparently reliable these measurement are difficult to be explained by the specific diseases but for the elevated HbA 2 measured in HIV patients treated with thymidine derivate, possibly influencing the expression of the d gene 15 (summarized in Table 1). …”

Section: Elevated Hemoglobin a 2 Occasionally Measured In Other Diseasesmentioning

confidence: 99%

HbA2 Measurements in β-Thalassemia and in Other Conditions

Ivaldi

Barberio

Harteveld

et al. 2014

Thalassemia Reports

View full text Add to dashboard Cite

show abstract

“…First stage: all samples are subjected to the single tube osmotic fragility test (in 0.36 % NaCl) and blood smear screening (to identify eventual morphological alterations of red cells). All samples that result to be positive for one or both tests undergo the second stage, consisting of complete blood count on an automatic counter, followed by separation and measurement of the haemoglobin fractions by high-performance liquid chromatography (Stephens et al 2012;Van Delft et al 2009). …”

Section: (3) Diagnostic Testsmentioning

confidence: 99%

Carrier screening for inherited haemoglobin disorders among secondary school students and young adults in Latium, Italy

Amato¹,

Cappabianca²,

Lerone³

et al. 2013

J Community Genet

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To reduce the incidence of β-thalassaemia major and other severe haemoglobin-related disorders by the early identification of healthy carriers, the Centro Studi Microcitemie Roma has been organising since 1975 a prevention programme in Latium, an Italian central region. This programme entails two different types of carrier screening on a voluntary basis: a universal screening offered to secondary school students and a screening offered to young adults. In 36 years of scholastic screening (from 1975 until 2011), 1,466,100 students have been examined and 26,786 (1.8 %) carriers of non-α thalassaemia have been identified. In the extra-scholastic screening, 388, 690 adult subjects (including the carriers' relatives) have been examined and a total of 38,457 (9.9 %) carriers of non-α thalassaemia have been detected. These results demonstrate that the precocious identification of healthy carriers allowed the identification of at-risk couples and reduced to zero the birth of affected babies in the Latium native population. This programme does not involve huge resources and is relatively inexpensive and, as such, it is essential to be offered to the total Latium scholastic and extra-scholastic population, which is epidemiologically changing due to migratory fluxes from countries in which haemoglobin disorders are common.

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“…4,5 Quantitative measurement of HbA2 can be performed by many techniques like cellulose acetate electrophoresis, micro column chromatography, capillary electrophoresis but high performance liquid chromatography (HPLC) is preferred in many laboratories. 6 In this case, this individual was asymptomatic for thalassemia trait. There was no previous history of anemia.…”

Section: Introductionmentioning

confidence: 72%

Report of Beta Thalassemia in Newar Ethinicity

Bhatt¹

2018

Ann. Clin. Chem. & Lab. Med.

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Beta thalassemia trait is a heterogeneous autosomal recessive form of beta thalassemia. Individual with beta thalassemia are clinically asymptomatic. Here we have described a case that has been incidentally diagnosed as beta thalassemia trait. A 31 year old male form Newar Community came to hospital for routine health checkup was send for hematological investigation. On examination, his red cell morphology was found to be microcytic hypochromic and his hemoglobin concentration was mildly decrease. His other parameters was evaluated and requested for analysis of iron profile and hemoglobin electrophoresis. Iron profile test was normal. Hemoglobin electrophoresis showed prominent band in HbA2 region. Presence of HbA2 band was confirmed by hemoglobin variant HPLC analysis. A diagnosis of heterozygous beta thalassemia trait was made. Prevalence of beta thalassemia gene in Tharu population was reported but its presence in other communities is still unknown therefore, it is recommended to study the prevalence of beta thalassemia gene in Newar ethnicity as well.

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ICSH recommendations for the measurement of Haemoglobin A₂

Cited by 97 publications

References 37 publications

HbA2 Measurements in β-Thalassemia and in Other Conditions

HbA2 Measurements in β-Thalassemia and in Other Conditions

Carrier screening for inherited haemoglobin disorders among secondary school students and young adults in Latium, Italy

Report of Beta Thalassemia in Newar Ethinicity

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ICSH recommendations for the measurement of Haemoglobin A2

Cited by 97 publications

References 37 publications

HbA2 Measurements in β-Thalassemia and in Other Conditions

HbA2 Measurements in β-Thalassemia and in Other Conditions

Carrier screening for inherited haemoglobin disorders among secondary school students and young adults in Latium, Italy

Report of Beta Thalassemia in Newar Ethinicity

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Product

Resources

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ICSH recommendations for the measurement of Haemoglobin A₂