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

Background Mycosis fungoides (MF) and Sézary 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...

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Published in:International journal of dermatology 2022-04, Vol.61 (4), p.442-454
Main Authors: Miyashiro, Denis, Sanches, José A.
Format: Article
Language:English
Subjects:
Age
Brazil - epidemiology
Cohort Studies
Demographics
Diagnosis
Female
Fungal infections
Humans
L-Lactate dehydrogenase
Lactate dehydrogenase
Lactic acid
Lymphoma
Male
Medical prognosis
Middle Aged
Multivariate analysis
Mycosis
Mycosis fungoides
Mycosis Fungoides - diagnosis
Mycosis Fungoides - epidemiology
Mycosis Fungoides - therapy
Neoplasm Staging
Patients
Prognosis
Public health
Retrospective Studies
Sezary Syndrome - diagnosis
Sezary Syndrome - epidemiology
Sezary Syndrome - therapy
Skin Neoplasms - diagnosis
Skin Neoplasms - epidemiology
Skin Neoplasms - therapy
Survival
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Summary:Background Mycosis fungoides (MF) and Sézary 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 São 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.
ISSN:0011-9059
1365-4632
DOI:10.1111/ijd.15865