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Actualizaciones en linfomas de la zona marginal

Marie Donzel1, Lucile Baseggio2, Camille Laurent3

  • 1Institut de pathologie multisite, hospices civils de Lyon, Lyon, France; Université Claude-Bernard Lyon 1, 69100 Villeurbanne, France; Centre international de recherche en infectiologie (CIRI), Institut national de la santé et de la recherche médicale (Inserm) U1111, Centre national de la recherche scientifique (CNRS), UMR5308, École normale supérieure de Lyon, Lyon, France.

Annales de pathologie
|February 20, 2026
PubMed
Resumen

Esta revisión cubre cinco subtipos de linfoma de la zona marginal (MZL), incluidas las formas pediátricas y cutáneas recién definidas. Destaca características compartidas y distintas, centrándose en datos moleculares para mejorar el diagnóstico.

Palabras clave:
linfoma de la zona marginal extraganglionarlinfoma de células B indolentelinfoma de células B indolentelinfoma de la zona marginallinfoma de la zona marginal extraganglionarlinfoma primario cutáneo de la zona marginallinfoma esplénico de la zona marginalproliferación linfoproliferativalinfoproliferaciónMALTlinfoma de la zona marginallinfoma primario cutáneo de la zona marginallinfoma esplénico de la zona marginal

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Área de la Ciencia:

  • Hematología
  • Oncología
  • Patología

Sus antecedentes:

  • Las clasificaciones de la OMS y la ICC de 2022 delinean cinco entidades de linfoma de la zona marginal (MZL).
  • Estas incluyen MZL extraganglionar (linfoma MALT), MZL nodal (NMZL), MZL esplénico (SMZL), MZL nodal pediátrico (PNMZL) y MZL cutáneo primario (PC-MZL).
  • Si bien comparten características comunes, cada subtipo de MZL exhibe características únicas.

Objetivo del estudio:

  • Revisar las entidades recién definidas de linfoma de la zona marginal nodal pediátrica (PNMZL) y linfoma de la zona marginal cutáneo primario (PC-MZL).
  • Discutir datos moleculares recientes relevantes para el diagnóstico de linfomas de la zona marginal.
  • Proporcionar una visión general actualizada de los subtipos de MZL reconocidos.

Principales métodos:

  • Revisión de literatura de clasificaciones recientes y estudios moleculares.
  • Análisis comparativo de características morfológicas, inmunofenotípicas y genéticas en subtipos de MZL.
  • Enfoque en entidades novedosas y avances diagnósticos.

Principales resultados:

  • Cinco entidades distintas de MZL son reconocidas, con PNMZL y PC-MZL siendo delineadas recientemente.
  • Existen características compartidas y distintas entre los subtipos de MZL, lo que impacta el diagnóstico.
  • Los datos moleculares emergentes ofrecen potencial para mejorar la precisión diagnóstica.

Conclusiones:

  • La clasificación precisa de los subtipos de MZL es crucial para el manejo adecuado del paciente.
  • La investigación adicional sobre características moleculares mejorará la precisión diagnóstica.
  • La revisión enfatiza la importancia de las clasificaciones actualizadas y las herramientas diagnósticas para MZL.