Fotoféresis en el tratamiento de Síndrome de Sézary: Caso clínico

• Montserrat Molgó ^[1] ; Alejandra Jaque ^[1] ; Verónica Vial ^[2] ; Mauricio Ocqueteau ^[1] ; Jaime Pereira ^[1] ; Mayling Chang ^[1] ; Sergio González ^[1]
  1. [1] Pontificia Universidad Católica de Chile

     Pontificia Universidad Católica de Chile

     Santiago, Chile

     
  2. [2] Universidad de Los Andes

     Universidad de Los Andes

     Colombia

     
• Localización: Revista Médica de Chile, ISSN-e 0034-9887, Vol. 143, Nº. 11, 2015, págs. 1449-1458
• Idioma: español
• Títulos paralelos:
  • Sézary syndrome treated with extracorporeal photopheresis: Report of one case
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• Resumen

  • Sézary syndrome (SS) is an unusually aggressive T- cell lymphoma characterized by the triad of erythroderma, the presence of more than 1,000 Sézary cells in peripheral blood and lymphadenopathies. It is accompanied by generalized pruritus and poor quality of life. The management of SS depends on its stage, patient comorbidities, and treatment availability. Extracorporeal photopheresis (ECP) is the first line of treatment for patients with T-cell lymphomas in stage IVA1, IVA2 or SS. This treatment comprises three phases: leukapheresis, photoactivation and subsequent reinfusion of lymphocytes. As it is an immunomodulatory therapy it does not produce generalized immunosuppression. We report a 76 year-old male with SS stage IIIb initially treated with 12 sessions of ultraviolet phototherapy without response. After 10 well-tolerated sessions of ECP, itching and skin lesions eventually disappeared.