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Trastornos linfoproliferativos de células NK / T impulsados por el EBV: diversidad clínica y conocimientos

Aleksander Luniewski¹, Sahil Chaudhary¹, Adam Goldfarb¹

¹Section of Hematopathology, Department of Pathology, University of Virginia Health, 1215 Lee Street, Charlottesville, VA 22903, USA.

Lymphatics

|February 9, 2026

Resumen

Los trastornos linfoides de células T / NK positivos para el virus Epstein-Barr (EBV) comparten vías moleculares, lo que sugiere un continuo de oncogénesis viral. Comprender estos perfiles moleculares ayuda a clasificar y tratar estas complejas condiciones.

Área de la Ciencia:

Hematología Hematología.
Oncología Oncología.
Virología Virología.

Sus antecedentes:

Las clasificaciones actuales de la OMS e ICC clasifican las neoplasias de células NK / T positivas para el EBV y los trastornos linfoproliferativos linfoides (LPD).
El perfil molecular revela interacciones complejas entre las proteínas del EBV, la inmunidad del huésped y las alteraciones genéticas en la patogénesis.
Existen distintos subtipos moleculares dentro de ENKTL (TSIM, MB, HEA), ANKL (mutaciones JAK/STAT, epigenéticas, TP53) y las recientemente definidas ENTNKL.

Objetivo del estudio:

Elucidar la patogénesis molecular y la diversidad de las neoplasias linfoides de células T/NK y LPDs positivas para el EBV.
Para explorar el panorama genómico de la infancia rara EBV impulsado por LPDs, incluyendo SMBA, HVLPD, y CAEBV.
Integrar datos clínicos, patológicos y moleculares para mejorar la clasificación y las estrategias de tratamiento.

Principales métodos:

Enfoques multiómicos para el perfil molecular de los subtipos de ENKTL.
Análisis de mutaciones en JAK/STAT, reguladores epigenéticos y vías TP53 en ANKL.
Secuenciación genómica de muestras de CAEBV y HVLPD, identificando mutaciones como DDX3X y KMT2D.

Principales resultados:

Las LPD de células NK/T del EBV+ exhiben diversidad molecular, con mutaciones específicas identificadas en ENKTL, ANKL y LPD infantiles.
Las muestras de CAEBV mostraron mutaciones conductor similares a las de ENKTL (DDX3X, KMT2D); las muestras de HVLPD mostraron mutaciones comunes de KMT2D y modificador de cromatina.
La secuenciación molecular para el SMBA y el linfoma sistémico de células T EBV positivo en la infancia es en gran medida incompleta.

Conclusiones:

Es probable que las LPD de células NK/T del EBV+ representen un continuo biológico impulsado por la oncogénesis viral.
La clasificación precisa requiere la integración de datos clínicos, patológicos y moleculares.
Este enfoque integrado puede conducir a una mejor estratificación del riesgo y un tratamiento personalizado para los pacientes.

Palabras clave:

EBV EBV, por sus siglas en inglés.Las células NK/T son células NK/T.El linfoma es un linfoma y el linfoma es un linfoma.Trastorno linfoproliferativo trastorno de la proliferación linfática.Las moléculas moleculares moleculares moleculares.

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