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

Abstract

Background Mycosis fungoides (MF) and S ezary 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 advancedstage 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. ConclusionsWe 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. European Organisation for Research and Treatment of Cancer (EORTC) classification of cutaneous lymphomas, MF is divided into classic MF (CMF), folliculotropic MF (FMF), pagetoid reticulosis (PR), and granulomatous slack skin (GSS). 2 Other clinicopathologic subtypes include erythrodermic (EMF), hypopigmented (HMF), poikilodermatous (PMF), granulomatous, papular, hyperpigmented, ichthyosiform, syringotropic, purpuric, interstitial, pustular, bullous, verrucous, and psoriasiform MF. 3 MF is an indolent disorder, and extracutaneous dissemination is rare. 1,4,5 SS presents with erythroderma, lymphadenopathy, and malignant cells in the blood. 1,6,7 Geographical variation in MF/SS characteristics is known. We aimed to analyze demographic, clinical, histopathological, immunopathological, molecular, laboratory, and follow-up characteristics of MF/SS patients in a Brazilian tertiary public health service. MethodsThis study, a single-center, retrospective cohort analysis, was approved by the local Institutional Review Board and followed the declaration of Helsinki. Patients with MF/SS evaluated at the University of São Paulo Medical School between 1989 and 2018 were included. Data were collected at diagn...

Mycosis fungoides and Sézary syndrome: clinical presentation, diagnosis, staging, and therapeutic management

“…In erythrodermic patients, the intense protein loss due to the universal skin alteration may cause hypoalbuminemia (126, 127). Hypoalbuminemia is more frequent in patients with advanced MF (20.9%) compared to early MF (5.4%), and in patients with SS (30.8%) compared to patients with MF (11.7%) (13). However, in multivariate analyses hypoalbuminemia was not associated with poorer prognosis in cutaneous lymphomas (13, 24, 57).…”

“…MF typically affects adults with 50 to 60 years at diagnosis, and the disease is rarely described in childhood and young adults, except in the hypopigmented subtype of MF, with median age at diagnosis of 32 years (2, 11-15). Seźary syndrome (SS) is a rare and aggressive variant of CTCL that typically affect the elderly with a median age at diagnosis between 60 and 65 years (3,4,10,13).…”

“…Pagetoid reticulosis and granulomatous slack skin are extremely rare and represent less than 1% (4). Among the other MF subtypes included in the classic MF group, poikilodermatous MF is the most frequent (10% to 11%) followed by hypopigmented MF (3% to 10%) (13,24).…”

“…On the other hand, a Brazilian study reported a frequency of 19.6%, and studies with the pediatric population report hypopigmented MF in up to 50% of the total number of patients analyzed. Thus, geographical variations in the frequency of hypopigmented MF occur according to the ethnic characteristics of the populations (13,15,61,66,67). Hypopigmented MF is characterized by hypopigmented patches on the trunk, thighs, buttocks, and extremities (Figure 5) (15).…”

“…When increased at the diagnosis, a poorer prognosis is observed in MF/SS (57). An increase in LDH is observed in 25% of MF patients (10,13,122,123).…”