ORIGINAL RESEARCH

Section: Medicine
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A Study on Hemoglobin Electrophoresis Profile in Patients with

Moderate to Severe Anemia
Bushra Khanam1, Sudhir Maurya2, Prakhar Gupta3, Shainila Shaikh4

nutritional anemias but not much has been studied about

ABSTRACT hemoglobinopathies in anemic patients especially in rural
Introduction: Hemoglobinopathy patients can benefit areas. Hemoglobinopathy patients can benefit temporarily
temporarily with nutritional supplementation and blood with nutritional supplementation and blood transfusions but
transfusions but long term outcome can be better if specific long term outcome can be better if specific diagnosis is made
diagnosis is made and specific therapy or precautions are and specific therapy or precautions are undertaken.2,3 We
undertaken. The aim of the study was to determine frequency carried out this study to determine frequency of hemoglobin
of hemoglobin disorders in patients presenting with moderate
disorders in patients who presented to us with moderate
to severe anaemia using hemoglobin electrophoresis.
to severe anemia with various manifestations and also to
Material and Methods: Indoor patients from departments
of internal medicine and paediatrics with moderate to severe
determine which disorder manifests more severely.
anemia (without other chronic illness), in a hospital in rural MATERIAL AND METHODS
area, were included in the study. Hemoglobin electrophoresis
The study was performed in a hospital in a rural area in Indore
was performed on them to evaluate for presence of
hemoglobin disorders. WHO reference range was taken for district of Madhya Pradesh on indoor patients in departments
anemia classification. of internal medicine and paediatrics. In total, 335 patients
Results: Out of 335 samples included in the study, 144 (43%) who had moderate to severe anemia were studied. Out of 335
were found to have hemoglobinopathies with sickle-beta patients, 162 were males and 173 were females. Age group
thalassemia being the most common occurrence followed varied from infants to middle-aged adults.
by beta-thalassemia carrier and sickle cell disorder. Beta- Study Design: The patients whose hemoglobin concentrations
thalassemia major was less common. Out of 144 patients with fell in the range of moderate to severe anemia and were not
hemoglobinopathies, 86 were males and 58 females. Mean having any other chronic illness were included in the study.
hemoglobin levels at presentation were also calculated and Hemoglobin range for anemia was taken according to WHO
beta-thalassemia presented with the lowest mean hemoglobin
recommendations1 (table-1).
(3.7 g/dl).
Hemoglobin electrophoresis was performed to identify
Conclusion: Recent statistics (NFHS-4) suggest that half
of the children and females and about one-fourth of males
variant and abnormal hemoglobins, including hemoglobin
in India are suffering from some degree of anemia. These A1 (HbA1), hemoglobin A2 (HbA2), hemoglobin S (HbS),
figures can have serious economic as well as health related hemoglobin F (HbF) and hemoglobin C (HbC).
implications, especially for a developing nation like India. The following reference range was taken4:
When properly managed, the outcome and quality of life • HbA1: 95%-98%
of patients with hemoglobin disorders can be improved and • HbA2: 1.5%-3.5%
since, a significant percentage of patients in our study were • HbF: < 2% (age-dependent)
found to have hemoglobinopathies of varying severity, it • HbC: Absent
would be advisable to perform investigations to determine • HbS: Absent
specific cause of anemia especially in rural areas where
supportive management stays the mainstay of treatment in RESULTS
most scenarios. Of the 335 samples tested for hemoglobin abnormalities,
144 (43%) were found to have hemoglobinopathies. Among
Keywords: Hemoglobin Electrophoresis, Moderate to Severe
Anemia
the ones with hemoglobinopathies, 86 (60%) were male
and 58 (40%) were female. Among the 162 males tested, 86
(53%) had hemoglobin disorders while among 173 females

INTRODUCTION
Recent most statistics (National Family Health Survey-4, Assistant Professor, 2Professor, 3PG Resident, 4PG Resident,
1

2015-16) suggest that 58% of children in 6-59 months of Department of Medicine, Index Medical College, Indore, India
age range, 53% of females and 22.7% of males in the age
group 15-49 years are anemic in India. For a developing Corresponding author: Sudhir Maurya, Professor, Department of
Medicine, Index Medical College, Indore, India
country like India, these figures have important health and
economic consequences, especially in rural areas. WHO How to cite this article: Bushra Khanam, Sudhir Maurya, Prakhar
reports suggest that about half of the world’s anemia is due Gupta, Shainila Shaikh. A study on hemoglobin electrophoresis
to nutritional deficiency, predominantly iron deficiency profile in patients with moderate to severe anemia. International
states.1 There have been numerous studies and surveys on Journal of Contemporary Medical Research 2018;5(3):C21-C24.

International Journal of Contemporary Medical Research C21

ISSN (Online): 2393-915X; (Print): 2454-7379 | ICV: 77.83 | Volume 5 | Issue 3 | March 2018
 Khanam, et al. Hemoglobin Electrophoresis Profile in Patients
Section: Medicine

Age Mild Moderate Severe 90

Patients
6- 59 month 10-10.9 7-9.9 <7 80
5-11 year 11-11.4 8-10.9 <8
70
12-14 year 11-11.9 8-10.9 <8
Female >14 years 11-11.9 8-10.9 <8 60
8-10.9 11-12.9 8-10.9 <8 50
Table-1: Haemoglobin concentration(g/dl) for diagnosis of 40 78
anemia and assesment of severtity acording to world health
organisation 30
20
33
Patients with Hemoglobinopathies 10 26
6 1
0
beta-thal beta-carrier Sickle cell HPHF Sickle beta
Figure-3: Graph representing the specific numbers of patients with
various hemoglobinopathies in our study
Female
40% 9
Male Average Hb (g/dL)
8
60% 7
6
5
4 8.1
200 7.3 7.2
3 6.3
2 3.7
150
1
76
115 0
100

50 86
58 Figure-4: Average hemoglobin concentrations of various
hemoglobinopathies in our patients
0
Male Female
60
Hb-Pathy Normal
Figure-1: Break-up according to gender 50
Number of Patients

2% 40

30
10%
beta-thal 20
8%
0% beta-carrier 10
Sickle cell
0
HpHF
57% <10 yrs 10-19 20-29 30-39 >40
23% Sickle beta
Age Group (yrs)
Normal Figure-5: Showing number of patients with various
hemoglobinopathies in different age groups.

(23%) followed by beta-thalassemia carrier and sickle cell

Figure-2: Results of hemoglobin electrophoresis disorder (including both trait and disease) being 10% and
8% respectively. Beta-thalassemia major was found in 2%
tested, 58 (33%) were positive for hemoglobinopathies of sample and 1 patient had Hereditary persistence of fetal
(figure-1). These results suggest that the likelihood of hemoglobin (HPHF) (figure-2).
hemoglobinopathies in severe anemia patients is higher Beta-thalassemia major patients presented with most severe
among males while females show a higher percentage of anemia with average hemoglobin concentration being just
anemias due to other causes, predominantly nutritional 3.7 g/dL. The patients with sickle-beta thalassemia had an
causes with iron deficiency being the most common culprit. average concentration of 6.3 g/dL while sickle cell disorder
Of the 335 samples tested, 191 were normal (57%) and had an average of 7.2 g/dL. Beta-thalassemia carrier patients
the most common disorder was sickle-beta thalassemia 78 had an average 7.3 g/dL and one with HPFH had hemoglobin

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International Journal of Contemporary Medical Research
Volume 5 | Issue 3 | March 2018 | ICV: 77.83 | ISSN (Online): 2393-915X; (Print): 2454-7379
Khanam, et al. Hemoglobin Electrophoresis Profile in Patients

Section: Medicine
8.1 g/dL (figure-3). the diseases and resulting in a population having higher
Most of the patients who presented with severe anemia and prevalence of sickle-beta thalassemia as compared to general
were diagnosed with hemoglobinopathy were less than 20 population. This is important particularly in this study as
years of age. Only 10 patients were more than 40 years of sickle-beta thalassemia was the most common hemoglobin
age (figure-4). defect observed in this study.
DISCUSSION Beta thalassemia syndromes are a group of hereditary
disorders resulting from genetic deficiency in the
Anemia is a major public health issue in India. National synthesis of beta-globin chains.16 In the homozygous state
Family Health Survey (NFHS-4 from 2015-16) showed (i.e., thalassemia major), it causes severe, transfusion-
that 58% of children in 6-59 months of age range, 53% of dependent anemia, whereas the heterozygous state (trait
females and 22.7% of males in the age group 15-49 years or thalassemia minor), causes mild to moderate microcytic
are anemic in India. In this observational study conducted anemia. Those presenting with clinical severity lying
among patients presenting with moderate to severe anemia between that of thalassemia major and minor are said to
as per WHO guidelines, a significant percentage of patients have thalassemia intermedia. Patients with thalassemia
were found to have various types of hemoglobinopathies. minor generally don’t require specific therapy whereas
This is of particular interest in rural areas where, even those with thalassemia major are transfusion dependent
today, specific laboratory investigations are not routinely and need iron chelation therapy. Splenectomy, allogenic
prescribed and such cases might go unnoticed or under- stem cell transplantation and supportive measures are also
treated. This study was conducted because of the observation required.
that most of the patients who had history of being diagnosed Sickle cell disease/anemia (SCD) and its variants are
with severe anemia recently or in the past were given blood
hereditary/genetic disorders resulting from the presence of
transfusions and/or iron supplements while ignoring the
a mutated form of hemoglobin i.e., hemoglobin S (HbS).16
need for finding out the actual cause for such degree of
Sickle cell disorders can cause significant morbidity and
anemia.
mortality. Morbidity, frequency of crisis, degree of anemia,
In India, females are more affected with anemia than
and the organ systems involved vary considerably from
males5,8,15 and the reasons range from high cost of healthcare
individual to individual. SCD is suggested by the typical
facilities, poor food quality and the low status of women.5
clinical picture of chronic hemolytic anemia and episodes
This condition becomes worse in rural areas as demonstrated
of vaso-occlusive crisis. Electrophoresis is used to confirm
by Kaur and Kochar6 in their study and also attributed poor
the diagnosis by showing presence of homozygous HbS and
nutritional status to lower hemoglobin levels in females.
can also document other hemoglobinopathies like HbSC,
A study from Central India also concluded that females
HbS-beta+ thalassemia. Management involves preventing/
have a higher prevalence of anemia than males especially
treating infections, management of vaso-occlusive crises,
those falling under moderate to severe category.7 Various
chronic pain syndromes, maintaining hydration, prevention
studies5,9,10 have indicated higher prevalence of malnutrition
of stroke and renal damage, managing chronic anemia etc.
among females than males especially in poor social-
and stem cell transplantation.
economic status populations. This suggests that causes other
Apart from various pharmacological and non-
than nutritional may be more important in male anemic
pharmacological measures for hemoglobin disorders, patient
patients and the results of this study also suggest the same as
education about the illness is also of paramount importance
53% of male patients had hemoglobin disorders.
especially counselling about various risks associated with
Central India and some other tribal areas of India are
the disease and measures to prevent marriages between at-
known to have a considerable degree of prevalence of
risk populations as it has been shown that supervision of at-
hemoglobinopathies.11,12,13 Sickle cell disorders as well as
risk population and conducting premarital genetic screening/
beta-thalassemias are especially prevalent in such areas. With
counselling results in reduction of burden of disease.2 This
proper and early diagnosis and providing specific treatment,
can only be made possible if proper investigations are
the severity of anemia in these disorders can be reduced as
carried out to determine cause of severe anemia and needed
well as frequency of complications can be brought down.2,3
measures are undertaken to eventually reduce the burden of
This is of significance in a developing nation like India where
the disease.
health issues can have major social and economic impact.
And since almost half of the population of India was found CONCLUSION
to be anemic, there is a need to address the approach towards As determined by the study, there’s a considerable chance
anemia related disorders. that the patients presenting with moderate to severe anemia
Beta-thalassemia and sickle cell anemia are both genetic/ can have some underlying genetic/hereditary disorder
hereditary disorders and are present in general population instead of nutritional causes of anemia. Therefore, steps are
in varying prevalence. Some populations/communities have needed to be taken for proper diagnosis and management
higher prevalence of these disorders due to preference to of patients with anemia especially in rural areas in order to
consanguineous marriages.15 Such trend has a possibility reduce burden of the disease as well as cost of treatment and
of breeding between populations having mutations for both general outcome of the patient.

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ISSN (Online): 2393-915X; (Print): 2454-7379 | ICV: 77.83 | Volume 5 | Issue 3 | March 2018
 Khanam, et al. Hemoglobin Electrophoresis Profile in Patients
Section: Medicine

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Volume 5 | Issue 3 | March 2018 | ICV: 77.83 | ISSN (Online): 2393-915X; (Print): 2454-7379