Linfoma primario cutáneo de células T epidermotropo agresivo CD8+ / Primary cutaneous aggressive epidermotropic CD8 + T-cell lymphoma
Dermatol. argent
; 27(1): [3-11], ene.-mar. 2021. il, tab
Article
in Spanish
| LILACS, BINACIS
| ID: biblio-1359875
Responsible library:
AR144.1
Localization: AR144.1; 616.5/25
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.
ABSTRACT
The aggressive epidermotropic CD8+ T-cell lymphoma or Berti's lymphoma, is a rare subtype of primary cutaneous lymphomas, first described in 1999. It is still considered a provisional entity by the latest World Health Organization classification of cutaneous lymphomas. Patients are commonly adults with a male predominance and it is characterized by widespread rapid evolving papules, hyperkeratotic annular plaques and ulcerated tumors. Histopathologicfindingsarecharacteristic,withaninfiltrate of medium-sized CD8+ atypical lymphocytes involving the epidermis with a pagetoid pattern. Ithasanaggressivebehaviorandtheprognosisispoor with a 5-yearsurvival less than 40%. Instead these disease represents a therapeutic challenge, the initial treatment consists on polychemotherapy and hematopoietic stem cell transplantation.
Full text:
Available
Collection:
International databases
Health context:
SDG3 - Target 3.8 Achieve universal access to health
/
SDG3 - Health and Well-Being
Health problem:
Delivery Arrangements
/
Target 3.8 Achieve universal access to health
Database:
BINACIS
/
LILACS
Main subject:
Skin Neoplasms
/
Lymphoma, Primary Cutaneous Anaplastic Large Cell
Limits:
Humans
Language:
Spanish
Journal:
Dermatol. argent
Journal subject:
Dermatology
Year:
2021
Document type:
Article
Affiliation country:
Argentina
Institution/Affiliation country:
Hospital General de Agudos Dr. Cosme Argerich/AR