Gamma/delta T-cell lymphoma with mycosis fungoides-like clinical course transforming to "T-cell-receptor-silent" aggressive lymphoma: Description of one case.
Aged
Biopsy
/ methods
Disease Progression
Fatal Outcome
Gene Rearrangement, delta-Chain T-Cell Antigen Receptor
/ immunology
Gene Rearrangement, gamma-Chain T-Cell Antigen Receptor
/ immunology
Humans
Immunophenotyping
/ methods
Lymphoma, Non-Hodgkin
/ complications
Lymphoma, T-Cell, Cutaneous
/ complications
Male
Mycosis Fungoides
/ pathology
Receptors, Antigen, T-Cell
/ immunology
Skin Neoplasms
/ pathology
T-Lymphocytes
/ pathology
cutaneous T-cell lymphoma
hematopathology
immunophetotypic
mycosis fungoides
primary cutaneous γδ T-cell lymphoma
Journal
Journal of cutaneous pathology
ISSN: 1600-0560
Titre abrégé: J Cutan Pathol
Pays: United States
ID NLM: 0425124
Informations de publication
Date de publication:
Sep 2021
Sep 2021
Historique:
revised:
28
04
2021
received:
24
01
2021
accepted:
12
05
2021
pubmed:
19
5
2021
medline:
29
12
2021
entrez:
18
5
2021
Statut:
ppublish
Résumé
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.
Substances chimiques
Receptors, Antigen, T-Cell
0
Types de publication
Case Reports
Langues
eng
Sous-ensembles de citation
IM
Pagination
1197-1203Informations de copyright
© 2021 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.
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