Fatal haemolytic transfusion reaction due to anti-Ena and identification of a novel GYPA c.295delG variant in a Thai family

Ploymanee Suwanwootichai; Genghis H Lopez; Morakot Emthip; Brett Wilson; Glenda M Millard; Sunisa Onpuns; Kanchana Laemsri; Sasitorn Bejrachandra; Yew-Wah Liew

doi:10.1111/vox.13358

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Fatal haemolytic transfusion reaction due to anti-Ena and identification of a novel GYPA c.295delG variant in a Thai family

Journal article

Peer reviewed

Fatal haemolytic transfusion reaction due to anti-Ena and identification of a novel GYPA c.295delG variant in a Thai family

Ploymanee Suwanwootichai, Genghis H Lopez, Morakot Emthip, Brett Wilson, Glenda M Millard, Sunisa Onpuns, Kanchana Laemsri, Sasitorn Bejrachandra and Yew-Wah Liew

Vox Sanguinis, Vol.117(11), pp.1327-1331

2022

DOI: https://doi.org/10.1111/vox.13358

PMID: 36102166

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Abstract

anti-Ena antibody

En(a-)

Ena (MNS28)

haemolytic transfusion reaction

MNS blood group system

Background and Objectives: High-frequency antigen Ena (MNS 28) is expressed on glycophorin A (GPA). En(a−) individuals can form anti-Ena when exposed to GPA. A Thai patient formed an antibody that reacted against all reagent red blood cells (RBCs). The patient received incompatible blood resulting in a fatal haemolytic transfusion reaction (HTR). This study aimed to characterize the antibody detected in the patient and investigate the cause of HTR. Materials and Methods: Blood samples from the patient and three of his family members were investigated. Massively parallel sequencing (MPS) and DNA-microarray were used for genotyping. Standard haemagglutination techniques were used for phenotyping and antibody investigations. Results: DNA sequencing showed the patient was homozygous for GYPA*M c.295delG (p.Val99Ter) predicting En(a−). Three family members were heterozygous for GYPA c.295delG. MPS and DNA-microarray predicted the patient was N− discordant with the N+ RBC phenotype. The patient's plasma was positive with enzyme/chemical-treated reagent RBCs but failed to react with En(a−) and MkMk RBCs. Conclusion: The GYPA c.295delG variant prevented GPA expression on RBCs resulting in En(a−) phenotype. The N+ phenotype result was probably due to the anti-N typing reagent detecting ‘N’ (MNS30) on GPB. The patient's alloantibody has anti-Ena specificity.

Details

Title: Fatal haemolytic transfusion reaction due to anti-Ena and identification of a novel GYPA c.295delG variant in a Thai family
Authors: Ploymanee Suwanwootichai (Corresponding Author) - Thai Red Cross Society
Genghis H Lopez (Author) - University of the Sunshine Coast, Queensland, School of Health and Behavioural Sciences - Legacy
Morakot Emthip (Author) - Thai Red Cross Society
Brett Wilson (Author) - Australian Red Cross Blood Service
Glenda M Millard (Author) - Australian Red Cross Blood Service
Sunisa Onpuns (Author) - Thai Red Cross Society
Kanchana Laemsri (Author) - Thai Red Cross Society
Sasitorn Bejrachandra (Author) - Thai Red Cross Society
Yew-Wah Liew (Author) - Australian Red Cross Blood Service
Publication details: Vox Sanguinis, Vol.117(11), pp.1327-1331
Publisher: Wiley-Blackwell Publishing Ltd.
Date published: 2022
DOI: 10.1111/vox.13358
ISSN: 1423-0410
PMID: 36102166
Organisation Unit: University of the Sunshine Coast, Queensland; School of Health and Behavioural Sciences - Legacy
Language: English
Record Identifier: 99676395902621
Output Type: Journal article

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Collaboration types: Domestic collaboration; International collaboration
Web Of Science research areas: Hematology

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