Diagnóstico y manejo del linfoma cutáneo de células T de tipo micosis fungoides y síndrome de Sèzary

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Artículo de revisión
Rev Asoc Colomb Dermatol. 2010;18: 205-217

Diagnóstico y manejo del linfoma cutáneo de células T de tipo micosis fungoides y síndrome de Sèzary

Diagnosis and treatment of cutaneous T cells lymphoma.
Autor(es): 
Oscar Jairo Valencia
mmvelasquez@yahoo.com
Médico y cirujano, Universidad de Antioquia; residente de Dermatología, Sección de Dermatología, Departamento de Medicina Interna, Facultad de Medicina, Universidad de Antioquia; Grupo de Investigación Dermatológica, GRID, Medellín, Colombia
Johana Marcela Pérez
Médico y cirujano, Universidad de Antioquia; Facultad de Medicina, Universidad de Antioquia, Medellín, Colombia
Margarita María Velásquez
Dermatóloga, doctor en Ciencias Básicas Biomédicas con énfasis en Inmunología; profesora, Sección de Dermatología, Departamento de Medicina Interna, Facultad de Medicina, Universidad De Antioquia; Grupo de Investigación Dermatológica, GRID, Medellín, Colo
Resumen: 

La micosis fungoides y el síndrome de Sèzary constituyen el grupo más frecuente de linfomas cutáneos de células T; tienen un curso lento y progresivo y un impacto negativo en la calidad de vida del paciente. En los estadios iniciales, la curación es anecdótica y en los casos avanzados pueden comprometer la vida del paciente; con las opciones terapéuticas actuales se consigue disminuir la sintomatología y se logran remisiones temporales. Para los estadios tempranos se propone el uso de terapias dirigidas a la piel, como los esteroides tópicos, la fotoquimioterapia PUVA y la radioterapia localizada, y otros no disponibles en nuestro medio, como la quimioterapia tópica y el bexaroteno, mientras que, para los estadios más avanzados, se recomiendan terapias que combinan las dirigidas a la piel con tratamientos sistémicos, como el interferón alfa, el vorinostat y la poliquimioterapia

Palabras clave: 
linfoma cutáneo de células T, micosis fungoides, síndrome de Sèzary, corticosteroides tópicos, fototerapia, Quimioterapia, radioterapia, interferón, inhibidores de la histona deacetilasa.

Summary: 

Mycosis fungoides and the Sèzary syndrome are the most frequent cutaneous T-cell lymphomas; they have a slowly progressive course and impact negatively the quality of life of patients. In early stages, healing is anecdotal and in advanced cases they may compromise patient's life. Current treatment options reduce the symptoms and achieve temporary remission. For early stages we propose the use of therapies targeted to the skin as topical steroids, PUVA photochemotherapy, and localized radiation therapy, and others not available in our environment such as chemotherapy and topical bexarotene, and for more advanced stages, combined therapies aimed to the skin with systemic therapies such as alpha interferon, and chemotherapy vorinostat.

Key words: 
lymphoma, T-cell, cutaneous, mycosis fungoides, Sèzary syndrome, topical steroids, phototherapy, chemotherapy, radiotherapy, interferon, histone deacetylase inhibitors.
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