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Approach In Managing Thalassaemia From Transfusion Perspective Dr. Nor Hafizah Ahmad Transfusion Medicine National Blood Centre 27 June 2019

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Page 1: Approach In Managing Thalassaemia From Transfusion Perspective · 2019-07-13 · Hb E Beta thalassemia on TDT regime (Transfusion dependent Thal) Juvenile SLE /Suspected panhypopitutarism

Approach In Managing Thalassaemia From Transfusion

Perspective

Dr. Nor Hafizah Ahmad

Transfusion Medicine

National Blood Centre

27 June 2019

Page 2: Approach In Managing Thalassaemia From Transfusion Perspective · 2019-07-13 · Hb E Beta thalassemia on TDT regime (Transfusion dependent Thal) Juvenile SLE /Suspected panhypopitutarism

Outline

Case Illustration

Introduction

Current situation In Malaysia

Malaysia Clinical Practice Guidelines

Risk of alloimmunization

Ensuring safety in multiply transfused patients

Conclusion

Page 3: Approach In Managing Thalassaemia From Transfusion Perspective · 2019-07-13 · Hb E Beta thalassemia on TDT regime (Transfusion dependent Thal) Juvenile SLE /Suspected panhypopitutarism

Case Illustration

13 years old ,Malay ,girl

Hb E Beta thalassemia on TDT regime (Transfusion dependent Thal)

Juvenile SLE /Suspected panhypopitutarism

Diagnosed in 2010, initially non transfusion dependent

Converted to TDT regime -Hb was persistently <7 with poor growth

No RBC phenotyping taken.

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Transfusion History

20 episodes of transfusion (April 2011 -September 2012) before converting to TDT

On regular 4 weekly transfusion since 2012

Mean HB in 2012 was 6-8g/dl, 2013 was 5-9g/dl and in 2014 4-6g/dl

Her spleen was increasing in size, despite being on a TDT regime.

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2013

difficulty getting compatible blood

DCT : 4+ for polyspecific AHG, IgG, C3d, Antibody screening : negative

July 2014

Transfusion requirements were more frequent since January 2014

She was pale- persistent low HB of 4-5g/dl requiring 2-3 weekly tx.

Repeat antibody identification :

DCT : 4+ for polyspecific AHG, IgG, C3d

Antibody identification: pan-agglutination ,non –specific auto-IgGAutocontrol positive 4+.

Page 6: Approach In Managing Thalassaemia From Transfusion Perspective · 2019-07-13 · Hb E Beta thalassemia on TDT regime (Transfusion dependent Thal) Juvenile SLE /Suspected panhypopitutarism

Case was referred to us in July 2014

Anti-C, Anti-Jkb, Auto-IgG, cold agglutinin. Elution showed Auto IgGspecificity.

Phenotype-

Difficulty in getting blood – presence of multiple alloantibodies with auto IgG

Page 7: Approach In Managing Thalassaemia From Transfusion Perspective · 2019-07-13 · Hb E Beta thalassemia on TDT regime (Transfusion dependent Thal) Juvenile SLE /Suspected panhypopitutarism

Mycoplasma test was positive

Connective tissue screening : C3/C4 and ANA were positive

No hx of prolong fever, hair loss, rash, and no joint pains.

Impression : Juvenile SLE with AIHA

Treated with Methylprednisolone for 3 days, Azathioprine 25mg od, Hydroxychloroquine 100mg od & weaned off to prednisolone 25mg od

Page 8: Approach In Managing Thalassaemia From Transfusion Perspective · 2019-07-13 · Hb E Beta thalassemia on TDT regime (Transfusion dependent Thal) Juvenile SLE /Suspected panhypopitutarism

December 2014

Back to monthly transfusion, less hemolysis

Hepatosplenomegaly, liver 5 cm and spleen 5 cm

2015

- developed multiple transfusion reaction

- Treatment were optimized ;methylprednisolone pre transfusion & IVIg

- Hb was better sustained

Page 9: Approach In Managing Thalassaemia From Transfusion Perspective · 2019-07-13 · Hb E Beta thalassemia on TDT regime (Transfusion dependent Thal) Juvenile SLE /Suspected panhypopitutarism

2016

- Developed flare of the disease

- Methylprednisolone for 3 days. Azathioprine was increased to 100mg od & dexamethasone was converted to prednisolone again

- Referred HKL for splenectomy

- Rituximab commence to control the hemolysis

2017 – regular monthly transfusion, on rituximab

2018

- Hemolyis not controlled with rituximab

- Referred to Hosp Ampang for exchange transfusion

- Patient became unstable, passed away in Ampang

Page 10: Approach In Managing Thalassaemia From Transfusion Perspective · 2019-07-13 · Hb E Beta thalassemia on TDT regime (Transfusion dependent Thal) Juvenile SLE /Suspected panhypopitutarism

Thalassaemia

Heterogenous group of genetic disorder

Reduced rate of synthesis of alpha or beta chain

Abnormal hemoglobin production leads to anaemia

Clinically patient is lethargic and pale, enlarged spleen, jaundice, dark urine, and failure to thrive

Required regular transfusion for survival

Page 11: Approach In Managing Thalassaemia From Transfusion Perspective · 2019-07-13 · Hb E Beta thalassemia on TDT regime (Transfusion dependent Thal) Juvenile SLE /Suspected panhypopitutarism

Hemoglobin Chain

In α-thalassemias, production of the α globin chain is affected

In β-thalassemia, production of the β globin chain is affected.

Page 12: Approach In Managing Thalassaemia From Transfusion Perspective · 2019-07-13 · Hb E Beta thalassemia on TDT regime (Transfusion dependent Thal) Juvenile SLE /Suspected panhypopitutarism

Clinical & Genetic Classiffication

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Clinically divided into :

a) Major : severe anaemia; no alpha or globin chain is produced. Cannot make hemoglobin

a) Intermedia : moderate anaemia with splenomegaly & iron overload

a) Minor/trait : mild anaemia; slight decrease in normal hemoglobin types

Page 14: Approach In Managing Thalassaemia From Transfusion Perspective · 2019-07-13 · Hb E Beta thalassemia on TDT regime (Transfusion dependent Thal) Juvenile SLE /Suspected panhypopitutarism

TRANSFUSION REQUIREMENT

http://www.haematologica.org/content/98/6/833

Page 15: Approach In Managing Thalassaemia From Transfusion Perspective · 2019-07-13 · Hb E Beta thalassemia on TDT regime (Transfusion dependent Thal) Juvenile SLE /Suspected panhypopitutarism

Widely distributed worldwide, more prevalent in certain areas especially among Asians, middle Mediterranean, Middle eastern & Far

Eastern populations (Singer et al, 2009.)

https://clinicalgate.com/hemoglobinopathies-structural-defects-in-hemoglobin/

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Thalassaemia In Malaysia

Most common hemoglobinopathies in Malaysia

Estimated 120-350 babies born with Thalassaemia each year (National Screening Program Malaysia, 2016)

Public heath issue ; psychological, social & economical Impact to patient, family and the nation

HbE and Beta- Thalassaemia are the most common inherited haematologic disorders affecting beta globin (Hassan et al, 2004)

Page 17: Approach In Managing Thalassaemia From Transfusion Perspective · 2019-07-13 · Hb E Beta thalassemia on TDT regime (Transfusion dependent Thal) Juvenile SLE /Suspected panhypopitutarism

Dr. Hishamshah Ibrahim/ MOH/2012

Page 18: Approach In Managing Thalassaemia From Transfusion Perspective · 2019-07-13 · Hb E Beta thalassemia on TDT regime (Transfusion dependent Thal) Juvenile SLE /Suspected panhypopitutarism

Malaysia Clinical Practice Guidelines

To minimise the likelihood of alloimmunisation,antigen matched blood for ABO, Rh and Kidd andany other antigen negative blood for defined antibody should be given.

Whenever possible, fresh blood of less than 14 days should be given.

Leucoreduced blood should be given to all thalassemia patients.

Page 19: Approach In Managing Thalassaemia From Transfusion Perspective · 2019-07-13 · Hb E Beta thalassemia on TDT regime (Transfusion dependent Thal) Juvenile SLE /Suspected panhypopitutarism

Alloimmunization In Thalassaemia

Transfusion therapy mainstay of treatment

Needs critical strategy to reduce morbidity & mortality

Risk of alloimmunization to minor red cell antigen which increased with repeated transfusion

No universal agreement or standards for most appropriate strategy for selection of red cells units

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Alloimmunization in ThalassaemiaRemains a major challenge in chronic transfusion therapy (Yazdanbakhsh, Ware et al. 2012)

Clinical importance as can lead to Hemolytic Transfusion Reaction (acute & delayed) with different risks of morbidity & mortality

Those who developed antibodies after few transfusion showed an increased risk of additional alloantibodies & haemolytic transfusion reaction. (Yazdanbakhsh, Ware et al. 2012)

Over the last 20 years risk of alloimmunization remains unchanged, while risk of transmissible infection had gone down (SHOT data , 2011)

Page 21: Approach In Managing Thalassaemia From Transfusion Perspective · 2019-07-13 · Hb E Beta thalassemia on TDT regime (Transfusion dependent Thal) Juvenile SLE /Suspected panhypopitutarism

Rate of alloimmunization

(Matteocci and Pierelli 2014)

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Risk of alloimmunizationa) Donor Factorsb) Host Factors

Page 23: Approach In Managing Thalassaemia From Transfusion Perspective · 2019-07-13 · Hb E Beta thalassemia on TDT regime (Transfusion dependent Thal) Juvenile SLE /Suspected panhypopitutarism

Risk Factors For Alloimmunization

Donor Factors

- Ethnic or racial disparity

- Homogenus population – lower rate compared to heterogenuspopulation

- Extended RBC antigen matching in donors

Host Factors- Duration of transfusion support

- Older age associated with increased rate of alloimmunization

- Splenectomy -significant risk, mechanism is unclear

- Alogeneic white blood cells – implicated in allergic & febrile reaction as well as alloimmunization

Page 24: Approach In Managing Thalassaemia From Transfusion Perspective · 2019-07-13 · Hb E Beta thalassemia on TDT regime (Transfusion dependent Thal) Juvenile SLE /Suspected panhypopitutarism

Study of Asian patient with Thalassaemia in a single community in US ;rate 20.8% becaouse only 5% of blood donors are Asian

Patients who had a splenectomy had a higher rate of alloimmunization than patients who did not have a splenectomy(36% vs 12.8%;P 5 .06).

Page 25: Approach In Managing Thalassaemia From Transfusion Perspective · 2019-07-13 · Hb E Beta thalassemia on TDT regime (Transfusion dependent Thal) Juvenile SLE /Suspected panhypopitutarism

Development of AntibodiesLower rate if first transfusion at < 2 years of age compared those who are transfused later (Gupta et al); < 3 years ( Spanos et al).

An immature immune system & some form of acquired immune tolerance to allogeneic red cells held responsible for the alloimmunization risk

Patient without spleen – promote enhancement of autoantibodies

(4.9 % Thompson et al, 11% Ameen et al, 21% Guirat-Dhouin et al)

Foreign blood not filtered due to absence of spleen. Patient without spleen – promote enhancement an autoimmune reaction

Page 26: Approach In Managing Thalassaemia From Transfusion Perspective · 2019-07-13 · Hb E Beta thalassemia on TDT regime (Transfusion dependent Thal) Juvenile SLE /Suspected panhypopitutarism

Autoimmunization in Thalassaemia

• Zimring et al explained non –exofacialpolymorphism (NEP) exists in some blood group

• NEP is in the cytoplasmic or transmembranedomains & not exposed on intact red cells –contribute to immunogenicity & provide mechanism for the development of autoantibodies

• A number of NEP identified in Kell, Kidd, Duffy

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Main antibody specificities

Thompson et al reported 697 patients the incidence is 21.2% in Caucasian & in Asian 13.3% with antibodies directed towards C,E,K antigens

In 382 Chinese patients, Cheng et al showed global red cell alloimmunizationanti-E (39.3%), anti Mia/Mur (30.85%), and anti-c (13%)

Saied et al reported from 95 Egyptian patients with 27 (28.4%) with anti-K and anti-E (23.6%)

In 58 patients, 8.6% of the patients had alloantibodies and 1.7% had autoantibodies. Alloantibodies identified were anti-E, anti-c, anti-K, anti-Jka, anti-N and anti-S ) (Noor Haslina, Ariffin et al. 2006)

Page 28: Approach In Managing Thalassaemia From Transfusion Perspective · 2019-07-13 · Hb E Beta thalassemia on TDT regime (Transfusion dependent Thal) Juvenile SLE /Suspected panhypopitutarism

Strategies For Prevention Of Alloimmunization

Baseline RBC phenotyping

Extended RBC matching

Leucocyte reduction

Enhanced Donor Recruitment

Page 29: Approach In Managing Thalassaemia From Transfusion Perspective · 2019-07-13 · Hb E Beta thalassemia on TDT regime (Transfusion dependent Thal) Juvenile SLE /Suspected panhypopitutarism

Baseline RBC phenotyping

- Prospective phenotyping

- Most blood bank offered serological method on recently transfused

- Recently transfused; DNA genotyping for RBC prediction

- Guide serological work in new antibody findings

- Prospective matching especially with multiple alloantibodies

- Not all centre practice; costs outweigh benefit

Page 30: Approach In Managing Thalassaemia From Transfusion Perspective · 2019-07-13 · Hb E Beta thalassemia on TDT regime (Transfusion dependent Thal) Juvenile SLE /Suspected panhypopitutarism

Extended RBC matching

-Reduction in alloimmunization expected with matched C,E,Kellphenotype (Singer, Wu et al. 2000)

- Minority programme ; include Duffy Kidd, MNS (extended phenotype)

- No consensus recommendation , not universally practiced

- Regional & national variability

- Depend on local inventory size & donor base for the region

Page 31: Approach In Managing Thalassaemia From Transfusion Perspective · 2019-07-13 · Hb E Beta thalassemia on TDT regime (Transfusion dependent Thal) Juvenile SLE /Suspected panhypopitutarism

Highly antigen matched available from donor of European background screened using molecular method. Found 96 units matched for ABO, Rh K (basic level)34 units if extended to Duffy (mid level)16 units if extended to Kidd & MNS (high level)

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Leucocyte reduction

Increasingly being used as universal procedure

Effect discordant (some says reduce alloimmunization, others say no difference)

Benefit -reduce febrile non hemolytic transfusion reaction

- Cytomegalovirus transmission

Page 33: Approach In Managing Thalassaemia From Transfusion Perspective · 2019-07-13 · Hb E Beta thalassemia on TDT regime (Transfusion dependent Thal) Juvenile SLE /Suspected panhypopitutarism

Enhanced Donor Recruitment

- In populations with non homogenus population ;

- Increase recruitment of donors

- Develop specific programme for donors

- Donors partner with specific patient ( Hillyer et al 2006)

- Recruitment of dedicated donor & limiting number of donors –effective strategy to reduce alloimmunization (Roberts et al, 2012, el-Danasoury et al 2012)

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Ensuring safety in multiply transfused patients ; Malaysian Perspective

• Ensuring adequate blood collection

• Improved safety of blood supply by using Nucleic Acid Testing

• Filtered /leucodepleted blood products

• Supplying phenotype matched blood

• Phenotyping/Genotyping blood donors

Page 35: Approach In Managing Thalassaemia From Transfusion Perspective · 2019-07-13 · Hb E Beta thalassemia on TDT regime (Transfusion dependent Thal) Juvenile SLE /Suspected panhypopitutarism

Phenotype Request Jun-Ogos 2015 according to diagnosis

Page 36: Approach In Managing Thalassaemia From Transfusion Perspective · 2019-07-13 · Hb E Beta thalassemia on TDT regime (Transfusion dependent Thal) Juvenile SLE /Suspected panhypopitutarism

Improved Safety by using Nucleic Acid Testing

• Thalassaemia patients at higher risks of acquiring transfusion transmitted infections.

• To reduce the risk, it is recommended that Nucleic Acid Testing (NAT) screening is expanded to cover the whole country.

• NAT introduced able to reduce window period

• Baseline screening result & 6 monthly screening

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Filtered /leucodepleted blood products

• As outlined by Thalassaemia Guidelines to use packed red blood cells depleted of leucocytes

• Reduce the incidence of patient developing non haemolytic transfusion reaction

• Reduce the risk of alloimmunization to HLA.

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Supplying Phenotype Blood

Perform full phenotyping for 15% of our yearly collection

Develop Rare Donor Registry Database

Strategy to recruit new rare donors:

• Mass Screening Project

- Target population eg; Thaipusam mobile, Kwan In Teng temple mobile

• Family screening

• Random phenotyping of daily blood collection

• This year - Embark collaboration with embassy

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Annual event UIA ‘Rare Donor Mobile’

Targeted rare phenotype- Fya-b-, SS ( African /Arab )- Jka-b- Indonesian students- Ind B neg, Bombay (Indian)

Page 40: Approach In Managing Thalassaemia From Transfusion Perspective · 2019-07-13 · Hb E Beta thalassemia on TDT regime (Transfusion dependent Thal) Juvenile SLE /Suspected panhypopitutarism

3346 (44.3%)

1937 (25.6%)

2266 (30.0%)

COMBINATION OF PHENOTYPE JAN-DEC 2018

3122 (41.4%)

4364(57.8%)

63 (0.8%)

Rarity Phenotyped Blood Supplied In Jan-Dec 2018

No. of Common Phenotype

No. of Moderate Rare Phenotype

No. Of Extreme Rare Phenotype

Total ‘18 Total 17

Common Phenotype

3122 (41.4%) 3303 (48.6%)

Moderate Phenotype

4364 (57.8%) 3450 (50.8%)

Extreme Phenotype

63 (0.8%) 44 (0.6%)

Total ‘18 Total 17

Single Phenotype 3346 (44.3%) 3210 (47.1%)

Double Phenotype

1937 (25.6%) 1758 (25.9%)

Multiple Phenotype

2266 (30.0%) 1838 (27%)

Syazana Akmal Sharifudin/PDN/2018

Page 41: Approach In Managing Thalassaemia From Transfusion Perspective · 2019-07-13 · Hb E Beta thalassemia on TDT regime (Transfusion dependent Thal) Juvenile SLE /Suspected panhypopitutarism

Donor Phenotype Study

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Classiffication of Donor Phenotype

Extremerely Rare Phenotype :

PP1Pk -, Ind b negative, Bombay, Parabombay , -D- , Jka-b-, RZRZ, Fya-b-, rh null, Yta negative, MAM negative, Doa.

Moderately Rare Phenotype :

Fya-, rr , SS, KK

Common Phenotype :

R1R1, Jka+b+, Fya+b+ Jka+, Jkb +, Fyb+

Page 43: Approach In Managing Thalassaemia From Transfusion Perspective · 2019-07-13 · Hb E Beta thalassemia on TDT regime (Transfusion dependent Thal) Juvenile SLE /Suspected panhypopitutarism

Molecular Diagnostics (Genotyping)

Benefits of molecular typing :

a) Solve discrepant serological results

b) Clearly define presence or absence of RBC antigens

c) Detect variants of weakly expressed antigen

d) Use when commercial antisera not available

d) Large-scale genotyping to screen donors

(Matteocci and Pierelli 2014)

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Molecular Typing In National Blood Centre

91 samples (71 patients and 20 donors) with unresolved results by serologic findings.

Molecular testing to confirm either subgroup, Rh D status or red cell genotyping.

Findings : 22 (24%) out of 91 samples showed discordance results between serological

and molecular testing.

The discrepancies were found in ABO blood group (12), Rh blood group (4) and red cell

genotypes (6) cases

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Red blood cell antigen genotyping platforms (ID-CORE XT) for Thalassaemia Patients in Sabah, Malaysia

Results were available for all 81 samples with ID CORE XT.

Out of 68 samples that had serological red cell phenotyping data, 31 samples (45.6%) were found to have discordant in at least one antigen result.

Most of the discordant is due to the weak expression of the antigen that was not detected by serological testing.

Syazana AS, Rozi HM , Nurul MY, NHafizah A, Shahnaz IS, Jamalluddin M, Saliz MZ

Page 46: Approach In Managing Thalassaemia From Transfusion Perspective · 2019-07-13 · Hb E Beta thalassemia on TDT regime (Transfusion dependent Thal) Juvenile SLE /Suspected panhypopitutarism

CONCLUSION

• RBC allosensitization remains a major challenge in transfusion dependent patient

• Ongoing research needed – mechanism of response

• Technologies- facilitate identification of patients at risk of alloimmunization

• Strategies to increase number of donors

• Serology remains gold standard

• Molecular genotyping is the way forward

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Page 48: Approach In Managing Thalassaemia From Transfusion Perspective · 2019-07-13 · Hb E Beta thalassemia on TDT regime (Transfusion dependent Thal) Juvenile SLE /Suspected panhypopitutarism

AcknowledgementDr. Noryati Abu Amin

Dr. Afifah bt Hassan

State Transfusion Specialists

Immunohematology Staffs

Rare Donor Registry Committee

Paediatric Department Hospital Teluk Intan

Special Thanks

Dato Dr Yasmin Ayob

Dato Roshida Hassan

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REFERENCES• Yazdanbakhsh, K., et al. (2012). "Red blood cell alloimmunization in sickle cell disease: pathophysiology, risk

factors, and transfusion management." Blood 120(3): 528-537.

• Matteocci, A. and L. Pierelli (2014). "Red blood cell alloimmunization in sickle cell disease and in thalassaemia: current status, future perspectives and potential role of molecular typing." Vox Sanguinis106(3): 197-208.

• http://www.haematologica.org/content/98/6/833

• Thompson AA, Cunningham MJ, Singer ST, et al.: Red cell alloimmunization in a diverse population of transfused patients with thalassemia Br J Haematol 2011; 153:121–128

• Schonewille H, Brand A: Alloimmunization to red blood cell antigens after universal leucodepletion. A regional multicentre retrospective study. Br J Haematol 2005; 129:151–156

• Cheng CK, Lee CK, Lin CK: Clinically significant red blood cell antibodies in chronically transfused patients: a survey of Chinese thalassemia major patients and literature review. Transfusion 2012; 52:2220–2224

• Sirchia, G., Zanella, A., Parravicini, A., Morelati, F., Rebulla, P. & Masera, G. (1985) Red cell alloantibodies in thalassemia major. Results of an Italian cooperative study. Transfusion, 25, 110–112. Spanos, T., Karageorga, M., Ladis, V., Peristeri, J., Hatziliami, A. & Kattamis, C. (1990) Red cell alloantibodies in patients with thalassemia. Vox Sanguinis, 58, 50–55.

• Noor Haslina, M. N., et al. (2006). "Red cell immunization in multiply transfused Malay thalassemic patients." Southeast Asian J Trop Med Public Health 37(5): 1015-1020.

• Singer, S. T., et al. (2000). "Alloimmunization and erythrocyte autoimmunization in transfusion-dependent thalassemia patients of predominantly Asian descent." Blood 96(10): 3369-3373.

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THANK YOU