Thalassaemia in Bangladesh by Dr. Waqar Ahmed Khan, MBBS, M.Phil who is Professor of Pathology, Bangladesh Institute of Child Health, Dhaka Shishu (Children) Hospital and President of Dhaka Shishu Hospital Thalassaemia, Dhaka, Bangladesh.

1. THALASSAEMIA IN BANGLADESH
Dr.
Dr Waqar Ahmed Khan MBBS M Phil
Khan.MBBS,M.Phil
Professor of Pathology
Bangladesh Institute of Child Health
Dhaka Shishu (Children) Hospital
President
Dhaka Shishu Hospital Thalassaemia
Dhaka,Bangladesh.

2. Bangladesh-D
B
Bangladesh-Demographic Data
l d h hi D t
 Area: 1,47,570 sq.km. or 57,295
sq.miles.
 Population: 142,319,000
 Birth rate:22.9 births/1000
population.
l ti
 Infant mortality:45/1000 live births
 Per capita income: US$ 1700
1700.
 6.1% of the total national budget is
allotted to health sector.
 Ethinic groups: Bengali 98%
 Tribal and Non –Bengali Muslims 2%
g

3. HEALTH SYSTEM
• Health services provided through public
and private sectors
p
• Public sector provides out patient,in
patient and preventive care
care.
• Private provides out patient, and in patient
curative care taking big share of profit

4. HEALTH SYSTEM
• Health,Nutrition & Population Sector
, p
Programme to reduce (2003-2011)
• 1 MMR
1.MMR
• 2.Infant and under 5 mortality rate
• 3.Malnutrition
• 4 Tuberculosis
4.Tuberculosis
• 5.Non-communicable diseases but not Hb
• disorders.
• Th l
Thalassaemia i lik l t b i l d d i th
i is likely to be included in the
next HNPSP

5. EPIDEMIOLOGY
• WHO Data
• Beta trait 3%
• Hb E trait 4%
• Our Data
• Beta trait 4.1%
• Hb E trait 6.1%

6. EPIDEMIOLOGY
• The most common abnormal Hb seen is
Hb E
Hb S and Hb H occasionally seen

7. EPIDEMIOLOGY
• Wide carrier variation in carrier status
• In Chittagong 48 tribal children were
screened 42% were E trait and
• Another screening programme in Bengali
school children(110) 15% were E trait and
10% were Beta trait
• I Gaibandha 2 B
In G ib dh 257 Bengali women were
li
screened and 22% were E trait and 10%
were Beta trait

8. EPIDIEMIOLOGY
Expected total number of beta thalassaemia
born annually -1040
y
Expected total number of Hb E b t
E t dt t l b f beta
thalassaemia born annually -6443

9. EPIDEMIOLOGY
• No data regarding the number of
g g
thalassaemic patients.
• There is no patient’s registry
patient s
• It is estimated that there about 90,000
patients
• 85% are Hb E beta thalassaemia
thalassaemia.
• There is no prevention programme at any
level.
• No regular awareness programme other
than on International Thalassaemia Day

10. Carrier status in different
divisions of Bangladesh(n-686)
Division
Di i i No tested
N t t d Beta trait
B t t it E t it
trait HbE di
dis S/D t it
trait
Dhaka 252 8(3.1) 13(5.2)
Chittagong 102 3(2.9)
3(2 9) 3(2.9)
3(2 9) 3(2.9)
3(2 9) 2(1.3)
2(1 3)
Khulna 84 2(2.4) 2(2.4)
Rajshahi 91 5(5.5) 15(16.5)
Sylhet 96 5(5.2) 4(4.2) I(1.0) 1(1)
Barisal 62 5(8.1) 5(8.0)
Total 687 28(4.1) 42(6.1)

11. Hb Elect/HPLC in Dhaka Shishu
Hospital 30/7/98-31/12/11
Hb ELECTROPHORESIS/HPLC 10928
NORMAL 5147
BETA THAL MAJOR 461
Hb E BETA THALASSAEMIA 1657
BETA TRAIT 2048
E TRAIT 1374
E DISEASE 148
S/D BAND 58

12. DIAGNOSTIC FACILITIES
• Cell counter widely available
• Hb Electrophoresis available in private
hospitals and l b t i i bi cities
h it l d laboratories in big iti
• HPLC in Dhaka S s u Hospital
C a a Shishu osp ta
Thalassaemia Centre.
• Limited prenatal diagnostic proced re
procedure
(amniotic fluid) and DNA analysis done in
Medical University

13. BLOOD TRANSFUSION
• General picture
• Voluntary non-rumeration blood 29 4%
non rumeration 29.4%
• Relative blood donor 60.5%
• Paid blood donor 10.4%
• Ref-Blood transfusion in Bangladesh
• M. D.Islam Asian J.Transfusion,2009;3(2)
•

14. BLOOD TRANSFUSION
• Blood Transfusion is not free of charge.
• It costs about $9.00 to purchase blood
• from blood bank centres.
• From thalassaemia centres with blood
bank facilities the cost is less.

15. Blood transfusion for
thalassaemic patients
• 60% of blood collected are utilised for
thalassaemic patients.
p
• Generally packed cells are used for blood
transfusion.
t f i
• Certain ce t es like Bangladesh
Ce ta centres e a g ades
Thalassaemia Samity and Red Crescent
Society use washed red cells
cells.
• Overall about 30% of patients can afford
to keep the targeted Hb level
l

16. Blood Transfusion
• Patients are also admitted in hospitals and
treated by blood transfusion in most public
y p
hospitals
• Patients in remote areas are deprived of
even these facilities

17. IRON CHELATORS
• All the three chelators are available in big
cities and imported .Government has
p
exempted tax on these drugs.
• The drugs are not available free of charge

18. DRUGS USED IN
BANGLADESH
CENTRE Deferiprone
D f i Desferroxami D f
D f i Deferasirox
i COMBINED
ne
Bangladesh 66% 34%
Thalassaemia
Samity
Thalassaemia 10% 60% 5% 25%
Welfare
Society
Bangladesh
Thalassaemia
Foundation
Dhaka Shishu 35% 15% 5% 45%
Hospital
Thalassaemia
Centre

19. Mutation analysis
The following results are obtained from 111 samples that
were sent to Dr Mary Petrou in the UK to observe the
type of mutations in Bangladesh.

20. Mutation pattern
IVS 1-5(G-C) 101 74.4%
Fr 41-42 (
41 42 (- 5 4.5%
4 5%
TTCT)
Fr-8-9(+G) 4 3.65
Codon 30(G-C) 4
30(G C) 2.7%
2 7%
-90(C-T)
90(C T) 3 2.7%
2 7%
Codon 15(G-A) 2
( ) 1.8%

21. Mutation
IVS 1-130(G-C) 2 1.8%
619bp deletion 2 1.8%
1 8%
Codon 30(G A) 2
30(G-A) 1.8%
1 8%
/Codon 16(-C)
( ) 1 0.9%
Codon 15(-T) 2 0.9%
121

22. Viral infection in multi-transfused
patients in DSHTC
No. of HbsAg HCV HIV
transfused positive Positive
patients
92 1(1.1%) 17(18.5%) Nil

23. Mymensingh Medical College and
Hospital
No of HbsAg HCV HIV
trransfused positive positive positive
patients
152 13.8% 12.5% NIL
Mollah et al,jn Health popul Ntr.2003

24. EXPERT THALASSAEMIA
CENTRES IN BANGLADESH
NAME NO
DHAKA SHISHU HOSPITAL THALASSAEMIA CENTRE 3000
BANGLADESH THALASSAEMIA SAMITY DHAKA
SAMITY,DHAKA, 3000+
BANGLADESH THALASSAEMIA FOUNDATION,DHAKA 796
THALASSAEMIA WELFARE SOCIETY,CHITTAGONG 700

25. Dhaka Shishu (Children)
Hospital

26. DHAKA SHISHU HOSPITAL THALASSAEMIA CENTER