Gamma/delta T‐cell lymphoma with mycosis fungoides‐like clinical course transforming to “T‐cell‐receptor‐silent” aggressive lymphoma: Description of one case

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 s...

Full description

Saved in:
Bibliographic Details
Published in:Journal of cutaneous pathology 2021-09, Vol.48 (9), p.1197-1203
Main Authors: Tomasini, Dario, Croci, Giorgio Alberto, Hotz, Annamaria, Cione, Stefania, Cecchetti, Caterina, Ciambelli, Fabrizio, Crivelli, Filippo
Format: Article
Language:English
Subjects:
Aged
Biopsy - methods
cutaneous T‐cell lymphoma
Disease Progression
Fatal Outcome
Fungal infections
Gene Rearrangement, delta-Chain T-Cell Antigen Receptor - immunology
Gene Rearrangement, gamma-Chain T-Cell Antigen Receptor - immunology
hematopathology
Humans
Immunophenotyping - methods
immunophetotypic
Lymphoma
Lymphoma, Non-Hodgkin - complications
Lymphoma, Non-Hodgkin - diagnosis
Lymphoma, Non-Hodgkin - metabolism
Lymphoma, Non-Hodgkin - pathology
Lymphoma, T-Cell, Cutaneous - complications
Lymphoma, T-Cell, Cutaneous - diagnosis
Lymphoma, T-Cell, Cutaneous - metabolism
Lymphoma, T-Cell, Cutaneous - pathology
Male
Mycosis
Mycosis fungoides
Mycosis Fungoides - pathology
Phenotypes
primary cutaneous γδ T‐cell lymphoma
Receptors, Antigen, T-Cell - immunology
Skin Neoplasms - pathology
T cell receptors
T-cell lymphoma
T-Lymphocytes - pathology
Citations: Items that this one cites
Items that cite this one
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary: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.
ISSN:0303-6987
1600-0560
DOI:10.1111/cup.14063