A histo‐immunopathologic and prognostic study of erythrodermic cutaneous T‐cell lymphoma

Abstract

Background Sézary syndrome (SS) and erythrodermic mycosis fungoides (E‐MF) represent two expressions of erythrodermic cutaneous T‐cell lymphoma (E‐CTCL). Methods Histopathologic features were compared on skin specimens from 41 patients with SS and 70 patients with E‐MF. Immunopathologic findings were compared on 42 SS and 79 E‐MF specimens. Results Specimens of SS usually showed band‐like dermal infiltrates with intermediate‐sized lymphoid cells and few plasma cells; on the other hand E‐MF more often had a perivascular infiltrative pattern, predominance of small/mixed lymphoid cells and eosinophils. SS also had lower numbers of CD8+ cells and higher numbers of CD62L+ cells compared to E‐MF. For E‐MF patients, the presence of large Pautrier collections, infiltrates with intermediate‐sized cells, increased number of mitotic figures and ≥50% CD62L+ cells in the dermal infiltrate correlated with a relatively poor disease‐specific survival. However, only the presence of mitotic figures retained prognostic significance with clinical stage as a covariate. Conclusions Clinical stage provides the most important prognostic information for patients with E‐CTCL. However, mitotic activity for E‐MF and CD8+ cells <20% for SS have additional value. We hypothesize that observed differences in plasma cell and eosinophil numbers may reflect the influence of CD62L+ central memory T‐cells in the microenvironment.

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

“…We observed Pautrier's microabscesses in 65.8%, but the literature demonstrates great variability (25-85%). 40,41 Lymphocyte atypia is an important finding. 42 However, in one-third of the cases it is absent, 43 similar to this study (present in 61.7%).…”

The Microenvironment’s Role in Mycosis Fungoides and Sézary Syndrome: From Progression to Therapeutic Implications

“…A suggested mechanism in which STAT3 activation in neoplastic T-cells leads to eosinophil accumulation in the TME through IL-5 production by malignant T-cells was hypothesized by Fredholm et al The authors speculated as to whether malignant T-cells may "trap" inactivated eosinophils by a high secretion of cyclooxygenase 2 and prostaglandin E2 (PGE2) [152]. Finally, a massive presence of eosinophils in the tumor infiltrate was observed in erythrodermic mycosis fungoides (E-MF) [153] and in follicular mycosis fungoides (F-MF) [154,155].…”

Pronostic des lymphomes T cutanés érythrodermiques

“…En 2019, Vonderheid et al (47) ont comparé les histologies de deux groupes de patients : ceux avec lymphome T érythrodermique et cellules de Sézary/µL >1000, et les patients ayant une érythrodermie sans critère hématologique. Ils ont mis en évidence comme facteur de mauvais pronostic un taux de cellules CD8+ inférieur à 20% des lymphocytes de l'infiltrat.…”