Silent T-cell receptor cutaneous T-cell lymphoma associated to a clonal plasma cell proliferation.
PTCL
gamma-delta T-cell lymphoma
silent TCR
Journal
Hematology reports
ISSN: 2038-8322
Titre abrégé: Hematol Rep
Pays: Switzerland
ID NLM: 101556723
Informations de publication
Date de publication:
19 Feb 2019
19 Feb 2019
Historique:
received:
21
08
2018
accepted:
07
12
2018
entrez:
28
3
2019
pubmed:
28
3
2019
medline:
28
3
2019
Statut:
epublish
Résumé
Within T-cell lymphomas (TCL) there are 2 entities expressing gamma-delta TCR: hepatosplenic gamma-delta T-cell lymphoma (HSGDTL) and the primary cutaneous gamma-delta T-cell lymphoma (PCGDTL). PCGDTL is a rare form of Tcell lymphoma with specific tropism for skin that have a dismal prognosis. Although even rarer, there have been reports of TCL with loss of expression of the TCR, which have been termed peripheral TCL TCR-silent type. We report the case of a cutaneous TCR-silent type lymphoma associated to a clonal plasma cell proliferation with an ominous outcome that led to a lot of discussion in its classification. Due to the aggressiveness of the disease and the scant evidence about therapy in this strange entity the outcome was fatal. We report a unique case of a TCR-silent cutaneous TCL with an exceptional histopathology, prolonged clinical evolution and a subsequent plasma cell clonal expansion.
Identifiants
pubmed: 30915203
doi: 10.4081/hr.2019.7841
pmc: PMC6397994
doi:
Types de publication
Journal Article
Langues
eng
Pagination
7841Déclaration de conflit d'intérêts
Conflict of interest: the authors declare no potential conflict of interest.
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