Background Subcutaneous panniculitis‐like T‐cell lymphoma (SPTL) as strictly defined by World Health Organization‐European Organization for Research and Treatment of Cancer classification is a rare cytotoxic α/β T‐cell lymphoma, characterized by primary involvement of subcutaneous tissue mimicking panniculitis. Objectives To describe the clinicopathologic, immunophenotypic, and molecular features of SPTL. Methods A 10‐year retrospective study of 18 patients diagnosed with SPTL was thoroughly reviewed according to clinicopathology, immunophenotype, and T‐cell receptor (TCR) gene rearrangement. Results Of the 18 patients, 16 patients were definitely diagnosed with SPTL. The median age was 26 years (ranged 14‐53 years) with female predominance. Most patients presented with prolonged fever and subcutaneous nodules and/or plaques, usually located on lower extremities. 37.5% of patients had hemophagocytic syndrome. The main histopathology was lobular panniculitis with rimming of atypical lymphocytes highlighted by CD3+, CD8+, Beta‐F1+, granzyme B+, and Ki‐67 (50%‐90%). Monoclonal TCR gene rearrangement was found in 50% of patients and upper extremities involvement indicated a poor prognosis. Conclusion The correlation between clinicopathologic and immunophenotypic study is the most helpful method to give a precise diagnosis of SPTL. Rimming of CD8+ atypical lymphocytes highlighted by high Ki‐67 index is highly specific for the diagnosis of SPTL.
Mycosis fungoides is the most common form of cutaneous T-cell lymphoma. Both large-cell transformed mycosis fungoides and mycosis fungoides bullosa are rare presentations and predict unfavorable prognosis. We report the case of a 61-year-old woman who presented with generalized erythematous scaly annular plaques, and histopathology confirmed the diagnosis of mycosis fungoides. She was treated with various conventional therapies but only achieved partial response and always relapsed after discontinuation of treatment. Her last treatment was combined chemotherapy (CHOP regimen) followed by romidepsin. However, 1 month after the last cycle of romidepsin, she developed multiple ulcerative masses and nodules. Skin biopsy was compatible with CD30+ large cell transformation, and she was treated with a new combination of chemotherapy (ifosfamide, carboplatin, etoposide). One day after receiving chemotherapy, multiple tense bullae on normal-appearing skin and mycosis fungoid plaques erupted. A histological study demonstrated subepidermal blistering with epidermotropism of atypical lymphocytes. Direct immunofluorescence study was negative. The results confirmed the diagnosis of mycosis fungoides bullosa. We present the first reported case of large-cell transformed mycosis fungoides coexisting with mycosis fungoides bullosa.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.
hi@scite.ai
10624 S. Eastern Ave., Ste. A-614
Henderson, NV 89052, USA
Copyright © 2024 scite LLC. All rights reserved.
Made with 💙 for researchers
Part of the Research Solutions Family.