Linfoma primario cutáneo de células T epidermotropo agresivo CD8+

Autores/as

  • María Belén Mariucci Vázquez Unidad de Dermatología, Hospital General de Agudos Dr. Cosme Argerich, Ciudad Autónoma de Buenos Aires, Argentina
  • Mariana Arias Unidad de Dermatología, Hospital General de Agudos Dr. Cosme Argerich, Ciudad Autónoma de Buenos Aires, Argentina
  • Alejandra Abeldaño Unidad de Dermatología, Hospital General de Agudos Dr. Cosme Argerich, Ciudad Autónoma de Buenos Aires, Argentina

DOI:

https://doi.org/10.47196/da.v25i1.2194

Palabras clave:

linfoma primario cutáneo, linfoma de Berti, epidermotropismo

Resumen

El linfoma de células T epidermotropo agresivo CD8+ o linfoma de Berti es un subtipo infrecuente de los linfomas primarios cutáneos, descripto en 1999. Aún se considera una entidad provisional en la última clasificación de linfomas cutáneos primarios de la Organización Mundial de la Salud. Predomina en los hombres adultos y se manifiesta con pápulas, placas hiperqueratósicas y tumores ulcerados, diseminados y de inicio agudo. En la histopatología, es característica la presencia de un infiltrado de linfocitos atípicos medianos CD8+, con epidermotropismo de patrón pagetoide. Tiene un comportamiento agresivo y es de mal pronóstico, con una sobrevida a los 5 años menor del 40%. Si bien esta entidad es un reto terapéutico, el tratamiento inicial se basa en poliquimioterapia y/o trasplante de células hematopoyéticas.

Biografía del autor/a

María Belén Mariucci Vázquez, Unidad de Dermatología, Hospital General de Agudos Dr. Cosme Argerich, Ciudad Autónoma de Buenos Aires, Argentina

Jefa de Residentes.

Mariana Arias, Unidad de Dermatología, Hospital General de Agudos Dr. Cosme Argerich, Ciudad Autónoma de Buenos Aires, Argentina

Médica de Planta. 

Alejandra Abeldaño, Unidad de Dermatología, Hospital General de Agudos Dr. Cosme Argerich, Ciudad Autónoma de Buenos Aires, Argentina

Jefa del Servicio.

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Publicado

2021-03-29

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Educación Médica Continua