RESUMO
BACKGROUND: Mycosis fungoides (MF) is the most common type of cutaneous T-cell lymphoma in adults and children. The prevalence has increased in some countries, but no descriptive studies of MF in the pediatric population have been done in Colombia to date. METHODS: A combined prospective-retrospective study of 128 patients with a diagnosis of MF confirmed by the dermatology department and dermatopathology laboratory of Universidad de Antioquia between 2008 and 2017. We describe the clinical and histopathologic variants, response to treatment, and progression of the disease in 23 patients under 18 years of age. RESULTS: The pediatric cases of MF accounted for 18% of all the cases on record. The median age of onset of lesions was 9 years, the median age at diagnosis was 11 years, and the median time between onset of lesions and diagnosis was 2 years. All patients were in early stages of the disease. Hypopigmented MF was the most common clinical presentation (in 52.2%), followed by classical MF (in 30.4%). Folliculotropic MF was identified in 17.4%. All patients were treated with topical corticosteroids and phototherapy. One patient received chemotherapy while still in the early stage of disease. Complete remission was achieved in 59.1% and a partial response in 40.9%. Only 2 patients remained asymptomatic for 5 years. CONCLUSION: We found hypopigmented MF to be the most common clinical presentation in patients under 18 years of age. The disease did not progress to advanced stages in any of the patients, although recurrence after treatment interruption was common.
Assuntos
Hipopigmentação/patologia , Micose Fungoide/patologia , Administração Tópica , Adolescente , Corticosteroides/administração & dosagem , Idade de Início , Criança , Pré-Escolar , Colômbia , Progressão da Doença , Feminino , Humanos , Hipopigmentação/tratamento farmacológico , Masculino , Micose Fungoide/tratamento farmacológico , Fototerapia , Estudos Prospectivos , Recidiva , Indução de Remissão , Estudos RetrospectivosRESUMO
Most knowledge about dendritic cells (DCs) and regulatory T cells in humans has been gathered from circulating cells but little is known about their frequency and distribution in lymphoid organs. This report shows the frequency, phenotype and location of DCs and regulatory T cells in deceased organ donors' spleens. As determined by flow cytometry, conventional/myeloid DCs (cDCs) CD11c(high)HLA-DR(+)CD123(-/low) were 2.3 +/- 0.9% and LIN(-) HLA-DR(+)CD11c(high) 2.1 +/- 0.3% of total spleen cells. Mature CD11c(high)HLA-DR(+)CD83(+) were 1.5 +/- 0.8% and 1.0 +/- 1.6% immature CD11c(high)HLA-DR(+)CD83(-) cDC. There were 0.3 +/- 0.3% plasmacytoid DCs (pDC) CD11c(-/low)HLA-DR(+)CD123(high) and 0.3 +/- 0.1% LIN(-)HLA-DR(+)CD123(high). Cells expressing cDCs markers, BDCA-1 and BDCA-3, and pDCs markers BDCA-2 and BDCA-4 were observed in higher frequencies than DCs with other phenotypes evaluated. CD11c(+), CD123(+) and CD83(+) cells were located in subcapsular zone, T cells areas and B-cell follicles. CD4(+)CD25(high) Tregs were 0.2 +/- 0.2% and CD8(+)CD28(-) comprised 11.5 +/- 8.1% of spleen lymphocytes. FOXP3(+) cells were found in T- and B-cell areas. The improvement in cell separation, manipulation and expansion techniques, will facilitate the manipulation of donor spleen cells as a part of protocols for induction and maintenance of allograft tolerance or treatment of autoimmune diseases.