RESUMO
T-cell lymphomas (TCLs) constitute a rare subset of non-Hodgkin lymphomas, with mycosis fungoides/Sézary syndrome (MF/SS) being the most common subtype of cutaneous TCLs (CTCLs). Considered an incurable but treatable disease, MF/SS management presents several challenges including diagnostic delays, debilitating effect on patients' quality of life, need for several lines of therapies, multidisciplinary care and cumulative drug toxicities limiting duration of use. The present review intends to provide an overview of the recent advances in our understanding of the biology of CTCL and how these are being leveraged to provide additional treatment options for management of advanced and recurrent disease. In addition, the discussion of the different modalities of treatment is summarised to further outline the importance of multidisciplinary care and early referral to CTCL centres.
Assuntos
Micose Fungoide/terapia , Síndrome de Sézary/terapia , Neoplasias Cutâneas/terapia , Corticosteroides/uso terapêutico , Idoso , Anticorpos Monoclonais/uso terapêutico , Antineoplásicos/uso terapêutico , Bexaroteno/uso terapêutico , Biomarcadores Tumorais/sangue , Ensaios Clínicos como Assunto , Terapia Combinada , Diagnóstico Tardio , Diagnóstico Diferencial , Elétrons/uso terapêutico , Transplante de Células-Tronco Hematopoéticas , Inibidores de Histona Desacetilases/uso terapêutico , Humanos , Interferon-alfa/uso terapêutico , Masculino , Micose Fungoide/diagnóstico , Micose Fungoide/patologia , Micose Fungoide/fisiopatologia , Estadiamento de Neoplasias , Células-Tronco Neoplásicas/química , Células-Tronco Neoplásicas/patologia , Terapia PUVA , Fotoferese , Prognóstico , Retinoides/uso terapêutico , Síndrome de Sézary/diagnóstico , Síndrome de Sézary/patologia , Síndrome de Sézary/fisiopatologia , Transdução de Sinais , Neoplasias Cutâneas/diagnóstico , Neoplasias Cutâneas/patologia , Neoplasias Cutâneas/fisiopatologia , Subpopulações de Linfócitos T/química , Subpopulações de Linfócitos T/patologiaRESUMO
Mycosis fungoides and Sézary syndrome are the most common of the cutaneous T-cell lymphomas, which are a heterogeneous group of neoplasms that affect the skin as a primary site. Although the aetiologies of mycosis fungoides and Sézary syndrome are unknown, important insights have been gained in the immunological and genetic perturbations that are associated with these diseases. Unlike some B-cell lymphomas, cutaneous T-cell lymphomas as a group are rarely if ever curable and hence need chronic-disease management. New approaches to treatments are being investigated and include biological and cytotoxic drugs, phototherapy, and monoclonal antibodies that are directed towards novel molecular targets. New molecular technologies such as complementary-DNA microarray have the potential to increase the accuracy of diagnosis and provide important prognostic information. Treatments can be combined to greatly improve clinical outcome without substantially increasing toxic effects in advanced disease that is otherwise difficult to treat. Although present treatment strategies are generally not curative, there is hope that experimental treatments, particularly immunotherapy, might eventually reverse or suppress the abnormalities of mycosis fungoides and Sézary syndrome to the point at which they become non-life-threatening, chronic diseases.