Characteristics and outcomes of 727 patients with mycosis fungoides and Sezary syndrome from a Brazilian cohort

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art_MIYASHIRO_Characteristics_and_outcomes_of_727_patients_with_mycosis_2022.PDF (450.21 KB)
Citações na Scopus
7
Tipo de produção
article
Data de publicação
2022
DOI
Scopus
Web of Science
PubMed
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ISSN da Revista
Título do Volume
Editora
WILEY
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Citação
INTERNATIONAL JOURNAL OF DERMATOLOGY, v.61, n.4, p.442-454, 2022
Projetos de Pesquisa
Unidades Organizacionais
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Resumo
Background Mycosis fungoides (MF) and Sezary syndrome (SS) are the most prevalent cutaneous lymphomas. They were not described in a large Brazilian cohort yet. We aimed, with this single-center, retrospective cohort analysis, to describe the characteristics and outcomes of MF/SS in a tertiary public health service in Brazil. Methods MF/SS patients evaluated at the University of Sao Paulo Medical School between 1989 and 2018 were included. Data were collected at diagnosis. Demographic, clinical, histopathological, immunopathological, molecular, laboratory, and follow-up data were analyzed. Results Among 727 patients, 92.6% (673) were diagnosed with MF, 7.4% (54) with SS. There were 51.2% (372) of males, 48.8% (355) of females. The median age was 51.8 years; it was higher in erythrodermic MF (60.2) and SS (60.9). Among MF, 41.8% (281) had classic MF, 4.9% (33) folliculotropic MF, 1.8% (12) granulomatous slack skin, and 0.3% (2) pagetoid reticulosis. Common subtypes included erythrodermic (14.1%, 95), hypopigmented (10.8%, 73), and poikilodermatous MF (10.8%, 73). Extracutaneous involvement was rare. Five, 10, 20, and 30-year overall survival rates were 97.3%, 92.4%, 82.6%, and 82.6% for early-stage, and 58.6%, 42.7%, 20.8%, and 15.4% for advanced-stage disease, respectively. After multivariate analysis, SS diagnosis, folliculotropic MF, erythrodermic MF, clinical stage, age (>= 60 years), increased lactate dehydrogenase, and large cell transformation conferred poorer prognosis. Conclusions We observed a higher percentage of hypopigmented MF compared to the literature, and demographic (older age) and prognostic (poorer prognosis) similarities between erythrodermic MF and SS, suggesting a possible relationship between these erythrodermic lymphomas. Factors associated with a poorer prognosis were compatible with the literature.
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https://observatorio.fm.usp.br/handle/OPI/45928
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Coleções
Artigos e Materiais de Revistas Científicas - FM/MDT
Artigos e Materiais de Revistas Científicas - HC/ICESP
Artigos e Materiais de Revistas Científicas - HC/ICHC
Artigos e Materiais de Revistas Científicas - LIM/53
Artigos e Materiais de Revistas Científicas - ODS/03