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Spectrum of Thal.pptx
1. Spectrum of Thalassemia and its
transfusion support in West Bengal.
Dr.Biplabendu Talukdar
Assistant Professor, Department of IHBT,MCH,kolkata
State Program Officer, State Blood Cell and HCP control program.
National Expert in Transfusion Medicine under DGHS,India
2. Introduction
• Thalassemia and Sickle Cell Disease are two common genetic
disorders that are chronic, life-restricting and require long and
specialized treatment.
• They cause distress and financial loss to the family and are a
great drain on the health resources of the country.
• India there are almost 42 million carriers of β-thalassemia,
around 1-1.5million patients of Thalassemia major.
• About 10,000 to 15,000 babies with β-thalassemia major are
born each year
3. Present scenario of West Bengal since 2007
Ser
ial
No
Name of
The
Institute
No of
Screened
% of
screened
each
individua
l unit
No of
Thalassemi
a carrier
detected
No of
Transfusion
Dependent
No of non
Transfusion
Dependent
No of
Sickle
No of TDT
infected
(HIV,HBV,
HCV)
No of
Hemophi
lia
1. Govt TCU
(24 in NO)
12,20,392 2.6% 215851
(17.69%)
18442
(1.5%)
24942 717 146+142+
1111
1192
2. NGOs (3 in
No)
4,50,928 9% 47499
(10.53%)
801
(0.18%)
594 136 0
Total 16,71,320 263350
(15.7%)
19243
(1.15%)
25491
(1.59%)
853 1399
(7.27%)
1192
4. Road Map
Antenatal Screening
Screening of Female school children from 15 years and above
Screening of spouse of Carrier .
Voluntary screening mandatory for college students.
Process of online registration
Voluntary Blood Donation at regular interval
5. • THALASSAEMIA CONTROL UNIT
• Out of total 34 TCUs, there are 2 State Level
Nodal Centres – (i) IHTM, MCH and (ii) NRS
MCH////
Sl.
No.
Nodal Officer-I, Director of IHTM, MC & H,
Kolkata.
Sl.
No.
Nodal Officer-II, HOD, Haematology, N.R.S. MC & H,
Kolkata.
1 IHTM, Medical College & Hospital 18 N.R.S. Medical College & Hospital
2 IPGMER-SSKM, Kolkata 19 R.G. Kar Medical College & Hospital
3 Burdwan Medical College & Hospital 20 C.N. Medical College & Hospital
4 Bankura Sammilani M C & H 21 North Bengal Medical College & Hospital
5 Malda Medical College & Hospital 22 Coochbehar Govt. MCH
6 Diamond Harbour Govt. Medical College &
Hospital
23 District Hospital Krishnanagar, Nadia
7 School of Tropical Medicine, Kolkata 24 District Hospital Jalpaiguri
8 Murshidabad MC & H 25 District Hospital, Balurghat, Dakshin Dinajpur
9 District Hospital, Suri, Birbhum 26 District Hospital, Tamluk, Purba Medinipur
10 Purulia Govt. MC & H 27 Uluberia S.D. Hospital, Howrah
11 Distirct Hospital, Hooghly 28 Alipurduar District Hospital
12 Barasat District Hospital, North 24 Pgs. 29 Kalna SD Hospital
13 Raiganj Government MCH, Uttar Dinajpur 30 Asansol District Hospital
14 Ranaghat SD Hospital 31 Kalimpong District Hospital
15 Jangipur SDH 32 Bishnupur District Hospital
16 Baruipur S.D. Hospital, South 24 Parganas 33 Contai S.D. Hospital
17 Basirhat District Hospital 34 Kharagpur S.D. Hospital
7. Thalassemia screening Flow chart
Antenatal mother visited at Hospital/RH/BH
1st
trimester
Counsellors of respective hospital Phlebotomist---- LT/MT of respective
Counselled units
Collection of 2 ml EDTA sample
in a vacutainer / preserve the sample with proper identification
sample send to nearest TCU with maintaining cold chain
testing of sample at TCU
screening positive screening negative
sending the report to respective centre
counselling by local person continue pregnancy
advice for testing of spouse
sample send to TCU
spouse screening positive spouse screening negative
advise for CVS /amniocentesis continue pregnancy
collection of CVS by trained person
CVS sample send to tertiary centre for molecular testing
CVS/amniocentesis sample
Positive Negative
Advice for Termination of Pregnancy continue pregnancy
Prepared by : Dr. Biplabendu Talukdar, SPO, HCP
MBBS,MD,M.Phil,MBA,PhD scolar
8. TRANSFUSION IN
THALASSEMIA
• The combination of transfusion and
iron chelation is now the Rx for
thalassemia.
• Combination gives a marked
improvement in survival , growth
,sexual development if started at the
right time.
11. REGIMEN
FOR
TRANSFUSIO
N
• Optimal time to start
transfusion?
• What red cell component is
indicated?
• How much blood to be
transfused?
• Transfusion interval?
• Transfusion efficiency?
• Adverse reaction during
transfusion?
12. Pre-requisites before transfusion therapy
• ABO blood grouping
• Rh( D) grouping with extended
phenotype ( C,c, E, e)
• Kell antigen
• Other – Kidd
Duffy
MNS etc
13. WHEN TO
BEGIN
TRANSFUSION
THERAPY
Patients should only begin transfusion
therapy once thalassemia has been confirmed
through laboratory diagnosis and molecular
studies
Hb levels are registered at less than 7g/dl on
two successive occasions, more than two
weeks apart.
Hb levels are >9.5g/dl but accompanying physical
characteristics are noted, such as: Poor growth, facial
and other bone deformities, fragile bones and bone
fractures , enlarged liver and spleen , impairment of
normal physical activities.
14. WHAT RED CELL COMPONENT?
Packed red cell
in additives.
Less than 15
days old RBC.
Phenotypically
matched RBC.
Leukodepleted
RBC.
BUFFY COAT
REDUCED.
WASHED RBC.
LEUKOFILTERED
RBC.
15. Management
All transfusion supervised by MOIC BB
and TCU
PRBC for all.
Phenotype matched (Rh and Kell )
Hospital level orientation for
Paediatrician, Orthopedic surgeons.
Team at tertiary centre for complicated
cases.
24 hours help line for all
16. West Bengal Blood Centre
• Total functional State Govt Blood Bank
in Kolkata 87 till date including 37
existing + 4 upcoming BCSU.
• Proposed BSU in West Bengal 78 ,
functional 36 in number,14 will be
started very soon and 28 under
construction .