RESUMEN
BACKGROUND: Limited data exist on patients with mycosis fungoides (MF) and Sézary syndrome (SS) from the southeastern United States, a region with a high proportion of African Americans (AA). OBJECTIVES: We sought to determine clinical characteristics, prognostic factors, and survival of patients with MF/SS in a southeastern US cohort, compare with other cohorts, and validate proposed revisions in MF/SS staging. METHODS: This was a retrospective chart review of patients from an academic dermatology referral center (Atlanta, GA) from 1998 to 2013. Kaplan-Meier estimates were calculated for overall survival, disease-specific survival, and progression; univariate and multivariate Cox proportional hazard models were used for assessment of prognostic variables. RESULTS: Of 393 patients, 55.2% were white, 43.3% AA, and 1.5% other; 52.7% were male and 47.3% female (ratio 1.1:1). Mean age was 53.6 years; mean age among AA was 48.9 years. In all, 19.6% died of disease; 21.9% experienced disease progression. Advanced TNMB classification, presence of a circulating clone without phenotypic evidence of blood involvement, and older age were predictors of poor disease-specific survival in the multivariate analysis, whereas AA race was not. LIMITATIONS: This study was from a single academic center. CONCLUSIONS: Outcomes of our patients generally paralleled those of other geographic regions. MF/SS may affect younger patients and more women than previously recognized, particularly among AA. Survival among AA may be more favorable than that observed in prior reports. Our data support the validity of the staging criteria revisions for MF/SS.
Asunto(s)
Micosis Fungoide/mortalidad , Micosis Fungoide/patología , Estadificación de Neoplasias/normas , Síndrome de Sézary/mortalidad , Síndrome de Sézary/patología , Neoplasias Cutáneas/mortalidad , Adolescente , Adulto , Negro o Afroamericano/estadística & datos numéricos , Distribución por Edad , Anciano , Anciano de 80 o más Años , Niño , Estudios de Cohortes , Progresión de la Enfermedad , Supervivencia sin Enfermedad , Femenino , Humanos , Estimación de Kaplan-Meier , Masculino , Persona de Mediana Edad , Análisis Multivariante , Micosis Fungoide/diagnóstico , Micosis Fungoide/etnología , Pronóstico , Modelos de Riesgos Proporcionales , Estudios Retrospectivos , Distribución por Sexo , Síndrome de Sézary/diagnóstico , Síndrome de Sézary/etnología , Neoplasias Cutáneas/diagnóstico , Neoplasias Cutáneas/patología , Sudeste de Estados Unidos/epidemiología , Análisis de Supervivencia , Resultado del Tratamiento , Adulto JovenRESUMEN
A total of 131 new cases of mycosis fungoides and Sézary syndrome were diagnosed clinically and histopathologically at our centre over a 5-year period. There were 87 males and 44 females with a mean age of 36.3 years (range 3-87 years) and no racial predilection. Of the 62 patients (47.3%) with classical mycosis fungoides, the majority were male (male : female = 4.2:1). There was one patient with Sézary syndrome. Patients aged older than 50 years were more likely to present with a longer duration of symptoms and advanced disease. In contrast to classical mycosis fungoides, the 47 patients diagnosed with hypopigmented mycosis fungoides had early stage disease, were younger, and no gender predilection was noted. The mean duration of follow up was 19.7 months (range 0.2-54.8 months). Complete remission was achieved in 24.7% and 53.8% of patients followed up at 1 and 3 years, respectively, using skin-directed and systemic treatment modalities appropriate for the stage of disease. There were five patients with progressive disease and three patients with advanced disease who died from disease-related complications. The most significant prognostic factors for 1-year and 3-year outcomes were the patient's duration of symptoms and stage of disease at presentation.