This document discusses transfusion support for children with thalassemia major. It notes that transfusion therapy should begin once thalassemia major is diagnosed based on clinical and laboratory findings. The goals of transfusion therapy are to maintain red blood cell viability and function, achieve appropriate hemoglobin levels, minimize effects of anemia, and avoid adverse reactions. The optimal regimen involves phenotype matched and leukoreduced red blood cells transfused every 3 weeks to maintain hemoglobin between 9-10.5 g/dL. Regular screening for transfusion-transmitted infections is also recommended.

1. Transfusion support in
Thalassemic child
Dr.Biplabendu Talukdar.
MD(Immuno-Hematology and Blood Transfusion)
M.Phil (Regenerative Medicine)
Consultant Peoples Blood Bank

2. THALASSEMIA
• Thalassemia means ‘the sea’ in the blood
• A genetic disorder (Autosomal recessive)
• Quantitative disorder of either α or β globin chain
of Hb.
• Classified into 3 groups according to clinical
manifestation: major, intermedia, minor.

3. Transfusion therapy should be
started as soon as a diagnosis of
thalassemia major has been
established both on clinical and
laboratory observation

4. Screening of Thalassemic Child
• Test for –
1.Hepatitis B surface Antigen,
If Test Negative – Hepatitis B vaccination before transfusion.
2. Serology for HIV – I and II
3. Serology for anti HCV
Regular screening of the above mention test at 6 month interval

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6. 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.

7. 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

8. GOALS OF TRANSFUSION
• Maintenance of red cell viability and function to ensure
sufficient transport of oxygen
• Achievement of appropriate haemoglobin level
• To minimise effects of anaemia and ineffective
erythropiesis while minimising iron overload
• Avoidance of adverse reactions, including transmission of
infectious agents.

9. REGIMEN FOR TRANSFUSION
• 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?

10. WHEN TO BEGIN TRANSFUSION
THERAPY
• Patients should only begin transfusion therapy once thalassemia
has been confirmed through laboratory diagnosis and molecular
studies and when:
• 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.

11. WHAT RED CELL COMPONENT?
• Packed red cell in additives.
• Less than 7 days old RBC.
• Phenotypically matched RBC.
• Leukodepleted RBC.
• BUFFY COAT REDUCED.
• WASHED RBC.
• LEUKOFILTERED RBC.

12. In the Peoples Blood Bank
• We provide Lekodepleted phenotype matched RBCs to the
patients within 5 days of collection.
• All cases antibody screening and gel cross-matching were
performed in blood bank.

13. Alloimmunization in different age groups and
gender

14. Contn…

15. Selection of Blood for transfusion in presence of
a specific alloantibody
• If an alloantibody identified by reagent cells-Antigen
negative blood unit should be issued for transfusion.
• Ex:- If an alloantibody against E antigen identified the
E antigen negative blood should be issued for
transfusion.

16. Frequency of Rh and Kell phenotypes
96.60% 92.08%
50%
18.91%
99.02%
0.79%
0.00%
20.00%
40.00%
60.00%
80.00%
100.00%
120.00%
D C c E e Kell
Study at MCH , Kolkata- Unpublished data

17. How much units to be cross-matched?
•Calculation: E-negative units (0.8) X c-negative units
(0.5)= 0.4
•If the blood group of patient: O with prevalence of 45%
•Then prevalence of compatible units = 0.4X0.45 = 1.8%
•By this way we can calculate the number of units to be
cross-matched to get a compatible unit in case of
multiple allo-antibodies.

18. Allo + Autoantibody
• When autocontrol & DAT both are positive along
with positive antibody screening -suspect
Autoantibody along with alloantibody
• Identify the allo-ab first and then issue an antigen
negative ‘best matched’ unit to the patient.

19. CALCULATING THE VOLUME OF
BLOOD NEEDED
• Transfusion regimes aim : Hb levels between 9-10.5g/dl
before transfusion
• Patients usually receive 10-15ml of concentrated red
blood cells per kg of body weight (volume), transfused
over 3-4 hours every 3 weeks interval.
• Patient suffering from cardiac problems or where blood
transfusions begin when levels of Hb are below 5g/dl,
smaller volumes of blood are administered, at a slower
rate 2-5ml of RBC/kg/hour.

20. ASSESSING THE EFFECTIVENESS OF A
BLOOD TRANSFUSION REGIME
• Measured in terms of the
rate of fall in levels of
haemoglobin.
• Should not exceed
1g/dl/week in
splenectomised patient
or
1.5g/dl/week in non-
splenectomised patients.

21. REASONS FOR INCREASE TRANSFUSION
REQUIREMENT
• Development of antibodies (alloimmunisation) to RBCs
• Enlarged spleen (hypersplenism) and/or liver
(hepatomegaly).
• Poor quality blood, meaning red blood cells have a shorter
lifespan and function less effectively
• Bleeding (e.g. from the gut)
• Increased red cell destruction from use of medication or
infection (e.g.malaria)
*Haemoglobin levels should ideally be measured before
and after every transfusion, in order to assess the
effectiveness of the treatment regime.

22. ADVERSE REACTIONS
• Febrile non-haemolytic transfusion reactions (FNHTR)-
can be prevented by leukofiltration.
• Allergic reactions - use wash RBCs
• Alloimmunization - prevented by using phenotype
matched RBCs and antibody screening before each
transfusion.
• Transfusion transmitted infections- Testing of HIV/Hepa-
B & C in blood bank by modern techniques.
Peoples Blood Bank is concerned for every thalassemia patients

23. IRON LOADING FROM TRANSFUSION
• 200mg iron present in one blood unit
• In Thal Major
•4 to 10 g of iron per year due to transfusion and
increase gut absorption.

24. Other management
•Bone marrow transplantation
•Neocyte transfusion
•Genetic Engineering
•Splenectomy

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