Vol 15, Issue 7, 2022
Online - 2455-3891
Print - 0974-2441
THE INCIDENCE OF BETA-THALASSEMIA MINOR IN PREGNANT FEMALES BY MEASURING
HBA2 THROUGH HIGH PERFORMANCE LIQUID CHROMATOGRAPHY
ANITA CHAUDHARY
1
, NINDER KUMAR
1
*, RITU KUNDAL
2
, RAMESH KUMAR
1
, PREET KAMAL SIBIA
3
1
Department of Pathology, Government Medical College, Patiala, Punjab, India.
2
Department of Peadiatrics, Maharishi Markandeshwar
Medical College, Solan, Himachal Pradesh, India.
3
Department of Obstetrics and Gynaecology, Government Medical College, Patiala,
Punjab, India. Email: drninder@gmail.com
Received: 04 April 2022, Revised and Accepted: 10 May 2022
ABSTRACT
Objectives: The aim of the study was to study the incidence of thalassemia minor by determining HbA2 levels in pregnant females attending Obstetrics
and Gynaecology OPD and HbA2 levels of husbands of positive females for beta-thalassemia trait.
Methods: The prospective study spanning over 1½ years was conducted in the Department of Pathology in 1020 pregnant females who attended the
Gynaecology and Obstetrics OPD of Government Medical College, Patiala for antenatal check-up. The pregnant females of any trimester without any
specific sign and symptoms whose Hb level was <10 g/dL were screened in the study. Levels of HbA2 and HbF were determined by high performance
liquid chromatography (HPLC) and the cases with raised HbA2 value above the cutoff limit (>3.5%) were labeled as BTT. Husbands of BTT positive
females were also screened for the trait. Incidence of all these cases was calculated and analyzed statistically.
Results: The majority of the females were in the age group of 21–30 years. In present study, we found that total 134 (13.1%) patients were having beta
thalassemia trait. Husbands of all these positive patients were also screened for BTT and only 2 (1.49%) of them were found to be positive.
Conclusion: HPLC has the advantage for screening and detection of various hemoglobinopathies by providing rapid and accurate results. HPLC can
detect and measure HbF and HbA2 in a single system. Early diagnosis and management of thalassemia can help in reduction of burden on society as
well as government.
Keywords: Beta thalassemia, Hemoglobinopathies, HbA2, HbF, High performance liquid chromatography.
INTRODUCTION
Human hemoglobin consists of two pairs of globin chains with
heme group attached to each of them. The individual globin chains
synthesized in postnatal life are designated as α, β, γ, and δ. HbA has
two α chains and two β chains (α2 β 2); HbF has two α chains and two
γ chains (α2 γ 2) and HbA2 has two α chains and two δ chains (α2 δ2).
Alpha chain synthesis is directed by two α genes, α 1 and α β, on
chromosome 16, and β and δ chain synthesis by single β and δ genes on
chromosome 11. γ chain synthesis is directed by two genes, G γ and A γ,
which are also located on chromosome 11 [1]. HbF is the predominant
hemoglobin of fetal life where as in children and adults, HbA is the
major hemoglobin. HbA2 and HbF are found in small quantities in adult
life (2–3.3% and 0.2–1.0%, respectively). The adult proportions of
hemoglobin A, A2, and F are usually attained by 6–12 months of age [1].
The disorders of hemoglobin synthesis can be grouped into three main
categories:
1. Due to structural variants of hemoglobin, such as HbS.
2. Due to failure of synthesis of one or more globin chains of hemoglobin
at a normal rate, as in thalassemias.
3. Those due to failure to complete the normal neonatal switch from
fetal hemoglobin to adult hemoglobin referred to as hereditary
persistence of fetal hemoglobin (HPFH) [1].
Thalassemias refer to heterogeneous group of disorders in which
there is decreased or absent synthesis of one or more polypeptide
chains (α or β) as a result of missense/non-sense mutations (single-
base substitutions) or frameshift mutations of the genes controlling
the structure of the hemoglobin protein chains in one or both
“allelic” globin genes causing decreased hemoglobin concentration,
microcytosis, and anemia [2]. The two major categories are the α and
β thalassemia while the others are rare forms. These subgroups have
in common an imbalanced globin synthesis and the globin produced in
excess is responsible for ineffective erythropoiesis and hemolysis. The
thalassemias result from the effect of a number of different molecular
defects leading to a variety of clinical and hematologic phenotypes.
Functionally, thalassemia mutations that cause a complete absence
of globin chain synthesis are called α0 or β0 thalassemias and that
cause reduced rate of synthesis of globin chains are called α+ or β+
thalassemias [3]. Clinically, the thalassemia is classified according to
their severity into major, intermediate, and minor forms [3].
Thalassemias are among the most common genetic disorders in the
world and an estimated 1.5% of the worldwide population is carrier
of β-thalassemia [4]. Thalassemia is mainly prevalent in South-east
Asia and Mediterranean countries. Due to high rate of migration and
inter racial marriage system, thalassemia is found all over the world in
recent years. About 10% of the total world thalassemia patients belong
to Indian subcontinent and among them 3–4% are carriers [5].
β thalassemia is the most common single gene disorder in India. More
than 30 million people worldwide carry thalassemia causing defective
gene. Carrier frequency varies from 3 to 17% in different populations [6].
Definite diagnosis of thalassemia can be made by a step-wise
algorithmic approach, starting with a detailed clinical history, through
hematologic evaluation complete blood count (CBC)], reticulocyte
count, red blood cell (RBC) morphology, Naked Eye Single Tube Red Cell
Osmotic Fragility Test (NESTROFT), protein based analytic methods
[Hb electrophoresis or isoelectric focusing (IEF), cation exchange high
performance liquid chromatography (HPLC), reversed phase HPLC]
© 2022 The Authors. Published by Innovare Academic Sciences Pvt Ltd. This is an open access article under the CC BY license (http://creativecommons.org/
licenses/by/4.0/) DOI: http://dx.doi.org/10.22159/ajpcr.2022v15i7.44840. Journal homepage: https://innovareacademics.in/journals/index.php/ajpcr
Research Article