Transfusion support in Thalassemic child

Dr.Biplabendu Talukdar.MD(Immuno-Hematology and Blood Transfusion)

M.Phil (Regenerative Medicine)Consultant Peoples Blood Bank

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.

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

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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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.

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

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.

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?

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.

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.

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.

Alloimmunization in different age groups and gender

Contn…

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.

Frequency of Rh and Kell phenotypes

D C c E e Kell0.00%

20.00%

40.00%

60.00%

80.00%

100.00%

120.00%

96.60% 92.08%

50%

18.91%

99.02%

0.79%

Study at MCH , Kolkata- Unpublished data

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.

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.

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.

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.

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.

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

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.

Other management

•Bone marrow transplantation•Neocyte transfusion•Genetic Engineering•Splenectomy

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THANK YOU