Vol. 2 No. 2, 2009 FOR Serial.pmd


Introduction:
The inherited diseases of haemoglobin are the most
common single gene disorders. With global improvement
in childhood diseases thalassaemia will become a major
health issue in millennium. The beta – thalassaemia is the
most common type of thalassaemia because they are so
common, occur widely in a broad belt ranging from
Mediterranean and parts of North and West Africa through
the Middle East, Bangladesh, India, Sri Lanka, Thailand
and other countries of South East Asia1.

 The hereditary disorders of haemoglobin are classified
into two broad groups, the thalassaemias and
haemoglobinopathies. The haemoglobinopathies are
characterized by the production of structurally defective
haemoglobin due to abnormalities in the formation of the
globin moiety of the molecule. The thalassaemias are
characterized by a reduced rate of production of normal
haemoglobin due to absent or decreased synthesis of one
or more types of normal globin polypeptide chains2.
Among more than 300 structural variants, Hb E is the
second most prevalent haemoglobin disorder in the world.

Haemoglobin E/Beta Thalassaemia- A Study in BSMMU
Md Abdul Aziz1, Masuda Begum2, Md. Sirajul Islam3, Naima Islam4, Md Jalilur Rahman5, Amin Lutful Kabir1

 1Assistant Professor, 2Associate professor, 5Professor, Chairman, Department of Haematology, BSMMU, 3Assistant Professor Department
of Haematology, Dhaka Medical College,4Assistant Professor Department of Haematology, National Institute of  Cancer Hospital,
Mohakhali,Dhaka.

Abstract
Background: Thalassaemias and haemoglobinopathies have been found sporadically in every ethnic group and
geographic region, they occur with particularly high frequency from the shores of the Mediterranean and Africa through
the Middle East, the Indian subcontinent, Burma and Southeast Asia. Objective: The study was designed to find out the
incidence of HbE/beta thalassaemia in BSMMU. Method: A total of 700 patients   suspected to have been suffering from
haemolytic anaemia were included in the study. Patients having evidence of haemolysis in peripheral blood film were
selected for reticulocyte count and haemoglobin electrophoresis in cellulose acetate membrane at pH 8.6. Result: The
study group of 700 patients underwent Hb-electrophoresis of which only 52 (7.4%) cases were diagnosed as HbE/beta
thalassaemia.   Out of 52 cases, 34 (65.4%) patients were found symptomatic and the remaining 18 (34.6%) patients were
asymptomatic. Out of 34 symptomatic cases of HbE/beta thalassaemia, only 14 cases needed blood transfusion.  Among
the 14 patients, only 8 patients needed more than 10 units of transfusion and 6 patients needed frequent transfusion that
is two units of blood in every month. Conclusion: It is clearly evident from the present and other studied so far carried
out in this Indian subcontinent and South-East Asia that hereditary haemolytic anaemia due to globin chain defects are
quite common in this region, especially in Bangladesh and are responsible for considerable morbidity and mortality. 

 Key words: Thalassaemia; Haemoglobinopathies; HbE/beta thalassemia.

[BSMMU J 2009; 2(2): 78-80]

Address for  Correspondence: Dr Md. Abdul Aziz, Assistant
Professor, Department of Haematology, BSMMU, Shahabag, Dhaka,
Email: aziz fcps@yahoo.com

Haemoglobin E is quite common in Bangladesh and has a
worldwide carrier of 53 millions1.  These may occur due to
continued migration of population from one area to another.

 The carrier of beta-thalassaemia trait is reported to be
more than 100 millions world wide3.

 In Bangladesh inherited haemoglobin disorders is quite
common but no definitive data regarding incidence of HbE/
beta thalassaemia. The aim of the study is to find out the
incidence of HbE/beta thalassaemia in BSMMU.

Methods:
This study was carried out in the department of
Haematology, BSMMU from January 2002 to December
2002. A total of 700 patients of both sex, suspected to
have been suffering from haemolytic anaemia were
included in the study.  Patients under 16 years of age, and
who were taking hydroxyurea and cytarabine were
excluded from the study.

Two ml venous blood was taken from each patient under
aseptic precautions and collected into EDTA bottles for
the estimation of haemoglobin concentration, complete
blood count with red cell morphology in peripheral blood
film stained by Leishman’s stain. Patients with evidence
of haemolytic feature in blood film were selected for

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reticulocytes count and haemoglobin electrophoresis in
cellulose acetate membrane at pH 8.6. 

52 patients diagnosed as HbE/beta thalassaemia, were
selected for the study population. They were thoroughly
interviewed regarding the age at presentation, family
history, presenting symptoms with duration of illness and
blood transfusion requirement.

Statistical Analysis was done using the SPSS 11.5. Results
were tested for level of significant using non-parametric
Chi-square (c2) test. ‘P’ value of < 0.05 was considered as
to be statistically significant at the level of 95% CI.

Results:

Among the 700 cases only 52 cases (7.4%) were diagnosed
as HbE/beta thalassaemia.

The mean ± SD with range of age of the study subjects
was 24.8 ± 6.1 with 16-48 yrs. Out of 52 patients 28 were
male (53.8%) and 24 female (46.2%) with a male: female
ratio 1.17:1 as shown in table I.

Table I
Sex distribution of the HbE/Beta thalassaemia subjects

(n=52)

Sex Frequency Percent Ratio
Male 28 53.8 Male: female1.17:1
Female 24 46.2
Total 52 100.0

34 (65.4%) patients were symptomatic and the rest (34.6%)
asymptomatic with statistical significance ( p = 0.027) . The
symptomatic patients presented with different symptoms
as shown in Table- II. 

Table II
Distribution of patients by Clinical presentation of

HbE/Beta thalassaemia subjects (n=52)

Clinical No. of Percentage P-value
presentation patients
Asymptomatic 18 34.6 0.027*

Symptomatic 34 65.4
Weakness 30 88.2
Pallor 22 64.7
Palpitation 14 41.2
Jaundice 11 32.4
Abdominal lump 06 17.4

* =p<0.05

On the basis of haemoglobin level 9 patients (17.3%) had
severe anaemia (Hb <6gm/dl), 24 (46.2%) moderate anaemia
(Hb 6-10 gm/dl) and 19 (36.5%) mild anaemia (Hb >10gm/
dl) with statistical significance ( p = 0.035) as shown in
table III.

Table III
Distribution of Patients by Severity of anaemia of HbE/

Beta thalassaemia (n=52)

Group No. of Percentage P-
(Hb: gmldl) patients value
Severe(<6) 09 17.3 0.035
Moderately severe (6-10) 24 46.2
Mild(>10) 19 36.5

Of the symptomatic cases, 13 patients (38.2%) needed red
cell transfusion (transfusion dependant) and 21 (61.8%)
did not ( transfusion independent) without statistical
significance ( p = 0.17)as shown in table IV.

Table IV
Distribution of Patients by Transfusion dependence of

HbE/Beta thalassaemia(n=34)

Blood No. of Percentage P-
transfusion needed patients value
Yes 13 38.2 0.17
No 21 61.8

Discussion:
The incidence of hereditary haemolytic anaemia in
Bangladesh is not known. However,  the data regarding
the incidence of hereditary haemolytic anaemia in some of
our neighbouring countries is available. In India, the
highest incidence of HbE trait has been reported from
West Bengal (3.9%), and it is also prevalent in Assam and
Tripura states4, 5. HbE/beta-thalassaemia is the commonest
of the thalassaemia syndrome in Myanmar7. Bangladesh
is in geographical continuity with West Bengal, Assam,
Tripura states of India and with Myanmar. The population
in West Bengal shares the common ethnic ancestry with
the people of our country.

Out of 700 patients taken initially in this study, only 52
cases of HbE/beta-thalassaemia were found which
constitutes 7.4%; and the rest 648 cases (92.6%) including
beta- thalassaemia trait, HbE disease, HbE trait and also
normal persons were not included in this study. This figure

BSMMU J Vol. 2, Issue 2, July 2009

79



slightly differs from another study6 where the frequency
of HbE/ beta-thalassaemia was 12.1%. This difference
between the two studies may be due to exclusion of
pediatric populations in the present study.

Examination of haemoglobin level in hereditary
haemoglobin disorders is a very good indicator of
measurement of severity of the disease. Other important
parameters for determining the severity of the disease are
the severity of sign and symptoms of anaemia.   In this
present study among the 52 patients only 34 patients were
symptomatic and 18 patients asymptomatic. Among all, 9
patients (17.3%) were severely anaemic, 24 (46.2%) patients
moderately anaemic and the rest 19 (36.5%) were only
mildly anaemic (Hb>10g/dl). These findings are similar to
the findings in United Kingdom1. Exactly the same types
of findings were noted by Aung – Thang – Batu et al7.
They also concluded that HbE/beta-thalassaemia is the
commonest of the thalassaemia syndromes presenting
with symptoms of anaemia in Myanmar.

Another parameter of measurement of severity of the
disease is transfusion dependency and frequency of
transfusion. Prawse Wasi and his co-workers have
systematically investigated the determinants for different
degrees of severity of anaemia in this group of patients.
They concluded that concomitant inheritance of an alpha-
thalassaemia 1 gene leading to elevated HbF level
responsible for the severity of anaemia8,9.

Conclusion:
It is clearly evident from the present and other studied so
far carried out in this Indian subcontinent and South-East
Asia that hereditary haemolytic anaemia due to globin
chain defects are quite common in this region, especially
in Bangladesh and are responsible for considerable
morbidity and significant mortality. These are the diseases
mainly of paediatric groups, adolescents and young adults.

Both genders are equally affected. Haemoglobinopathies,
particularly HbE and beta-thalassaemia are prevalent in
this country. When HbE co-exists with beta-thalassaemia
in the same individual, severe anaemia is manifested. The
large numbers of asymptomatic patients is also hidden
among the apparent normal population and are the real
threat to our future generation because of the possibility
of homozygous or double heterozygous inheritance or
silent spread of traits through marriage.

References:
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Synthesis. In A.V. Hoffbrand, Lewis MS, Tuddenham, editors.
Postgraduate Haematology 5 th  ed. Oxford: Butterworth
Henimann 2005; p85 –103.

2 .   Firkin F, Chesterman C, Penington D, Rush B. de Gruchy,s
clinical haematology in medical practice, 5th edition, 1989, 7:
137 – 171.

3 . Bessmann JD, Fein Stein DI. Quantitative anisocytosis as a
discriminant between iron deficiency and thalassaemia. Blood
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4 . Gupta SC, Methrota TN, Methrota VG. Haemoglobin E-
Thalassaemia in Uttar Pradesh. Indian J of Medical Res 1970;
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5 . Mitra SS, Kambo BS. Frequency of febrile illness in HbE-
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– 82.

6 . Haque MS, Alam MA, Khan Wa, Amin SK, Banu B, Hossain et
al. Thalassaemia situation in Dhaka Shishu Hospital. DS
(Child) HJ 1999; 15: 30 – 36.

7 . Michael EJ Beard, Thomas F, Necheles and Donald M. Allen.
Intensive Transfusion Therapy in Thalassaemia Major.
Paediatrics 1967; 40: 911-915

8 . Higgs. DR, Vickers.M.A, Wilkie AO, Pretorious IM, Jarman
AP and Weatherall DJ. A review of molecular genetics of the
human  α- globin gene cluster. Blood, 1989; 73: 1081-1104.

9 . Was P, Poortrakul P, Fucharoen S, Winichagoon P, Wilairant
P, Proomboon A. Thalassaemia in South – East Asia;
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Haemoglobin E/Beta Thalassaemia- A Study in BSMMU Md Abdul Aziz et al

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