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Gamma/delta T‐cell lymphoma with mycosis fungoides‐like clinical course transforming to 'T‐cell‐receptor‐silent' aggressive lymphoma: Description of one case

Authors :
Annamaria Hotz
Fabrizio Ciambelli
Stefania Cione
Giorgio Alberto Croci
Caterina Cecchetti
Filippo Crivelli
Dario Tomasini
Source :
Journal of Cutaneous Pathology. 48:1197-1203
Publication Year :
2021
Publisher :
Wiley, 2021.

Abstract

Primary cutaneous γδ T-cell lymphomas (PCGDTLs) are a heterogeneous group of lymphomas representing about 1% of primary cutaneous T-cell lymphomas (CTCLs) and mostly regarded as clinically aggressive. Current WHO-EORTC classification recognizes different clinic-pathologic subsets of PCGDTL, but it suggests that cases showing a mycosis fungoides (MF)-like clinical presentation and histopathology should be classified as MF irrespective of phenotype for their indolent course. Herein, we describe a case of γδ-MF, featuring at onset a granulomatous pattern, with subsequent clinical worsening signaled by the development of an ulcero-necrotic lesion and systemic dissemination, leading to death in 5 months. Clinical progression was sustained by a shift to mature T-cell lymphoma composed of medium to large-sized blastoid T-cells featuring a T-cell receptor (TCR) silent immunophenotype.

Details

ISSN :
16000560 and 03036987
Volume :
48
Database :
OpenAIRE
Journal :
Journal of Cutaneous Pathology
Accession number :
edsair.doi...........6d1916ca32b6785caf7c677a13202942
Full Text :
https://doi.org/10.1111/cup.14063