Development of EBV Related Diffuse Large B-cell Lymphoma in Deficiency of Adenosine Deaminase 2 with Uncontrolled EBV Infection.
J Clin Immunol
; 44(5): 118, 2024 May 17.
Article
en En
| MEDLINE
| ID: mdl-38758417
ABSTRACT
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.
Palabras clave
Texto completo:
1
Banco de datos:
MEDLINE
Asunto principal:
Adenosina Desaminasa
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Linfoma de Células B Grandes Difuso
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Herpesvirus Humano 4
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Infecciones por Virus de Epstein-Barr
Límite:
Female
/
Humans
/
Male
Idioma:
En
Revista:
J Clin Immunol
Año:
2024
Tipo del documento:
Article
País de afiliación:
Australia