Development of EBV Related Diffuse Large B-cell Lymphoma in Deficiency of Adenosine Deaminase 2 with Uncontrolled EBV Infection.


Journal

Journal of clinical immunology
ISSN: 1573-2592
Titre abrégé: J Clin Immunol
Pays: Netherlands
ID NLM: 8102137

Informations de publication

Date de publication:
17 May 2024
Historique:
received: 27 11 2023
accepted: 17 04 2024
medline: 17 5 2024
pubmed: 17 5 2024
entrez: 17 5 2024
Statut: epublish

Résumé

Deficiency of Adenosine Deaminase 2 (DADA2) patients presenting with primary immunodeficiency are at risk of uncontrolled EBV infection and secondary malignancies including EBV-related lymphoproliferative disorders (LPD). This paper describes the first case of EBV related diffuse large B-cell lymphoma in a patient with DADA2 and uncontrolled EBV infection. Consideration should be given to monitoring for EBV viraemia and to preventative EBV specific therapy in DADA2 and patients with at risk primary immunodeficiencies. A type I interferon (IFN) gene signature is associated with DADA2 though its association with immune dysregulation is unclear.

Identifiants

pubmed: 38758417
doi: 10.1007/s10875-024-01712-x
pii: 10.1007/s10875-024-01712-x
doi:

Substances chimiques

ADA2 protein, human 0

Types de publication

Case Reports Journal Article

Langues

eng

Sous-ensembles de citation

IM

Pagination

118

Informations de copyright

© 2024. The Author(s).

Références

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Auteurs

Logan S Gardner (LS)

School of Public Health and Preventive Medicine, Monash University, Clayton, Australia. logan.gardner@monash.edu.
ICPMR, NSW Health Pathology, Westmead Hospital, Sydney, Australia. logan.gardner@monash.edu.
Department of Immunology, Westmead Hospital, Westmead, Sydney, Australia. logan.gardner@monash.edu.

Lachlin Vaughan (L)

ICPMR, NSW Health Pathology, Westmead Hospital, Sydney, Australia.
The Lowy Cancer Research Centre, Kensington, Australia.

Danielle T Avery (DT)

Garvan Institute of Medical Research, Darlinghurst, Australia.

Isabelle Meyts (I)

Department of Immunology and Microbiology, Laboratory for Inborn Errors of Immunity, Department of Pediatrics, University Hospitals Leuven and KU Leuven, Leuven, EU, Belgium.

Cindy S Ma (CS)

Garvan Institute of Medical Research, Darlinghurst, Australia.
Clinical Immunogenomics Research Consortium of Australasia (CIRCA), Darlinghurst, Australia.
Faculty of Medicine and Health, St Vincent's Clinical School, UNSW Sydney, Sydney, Australia.

Stuart G Tangye (SG)

Garvan Institute of Medical Research, Darlinghurst, Australia.
Clinical Immunogenomics Research Consortium of Australasia (CIRCA), Darlinghurst, Australia.
Faculty of Medicine and Health, St Vincent's Clinical School, UNSW Sydney, Sydney, Australia.

Winny Varikatt (W)

ICPMR, NSW Health Pathology, Westmead Hospital, Sydney, Australia.
Faculty of Medicine, University of Sydney, Camperdown, Australia.

Ming-Wei Lin (MW)

ICPMR, NSW Health Pathology, Westmead Hospital, Sydney, Australia.
Faculty of Medicine, University of Sydney, Camperdown, Australia.
Clinical Immunogenomics Research Consortium of Australasia (CIRCA), Darlinghurst, Australia.

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