Peripheral T-cell lymphoma with t(6;14)(p25;q11.2) translocation presenting with massive splenomegaly.

Détails

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Version: Final published version
Licence: Non spécifiée
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ID Serval
serval:BIB_19B29CF3637B
Type
Article: article d'un périodique ou d'un magazine.
Sous-type
Etude de cas (case report): rapporte une observation et la commente brièvement.
Collection
Publications
Institution
Titre
Peripheral T-cell lymphoma with t(6;14)(p25;q11.2) translocation presenting with massive splenomegaly.
Périodique
Virchows Archiv
Auteur⸱e⸱s
Somja J. (co-premier), Bisig B. (co-premier), Bonnet C., Herens C., Siebert R., de Leval L.
ISSN
1432-2307 (Electronic)
ISSN-L
0945-6317
Statut éditorial
Publié
Date de publication
2014
Peer-reviewed
Oui
Volume
464
Numéro
6
Pages
735-741
Langue
anglais
Notes
Publication types: Case Reports ; Journal ArticlePublication Status: ppublish PDF: Case Report
Résumé
Recurrent chromosomal translocations associated to peripheral T-cell lymphomas (PTCL) are rare. Here, we report a case of PTCL, not otherwise specified (NOS) with the karyotype 46,Y,add(X)(p22),t(6;14)(p25;q11) and FISH-proved breakpoints in the IRF4 and TCRAD loci, leading to juxtaposition of both genes. A 64-year-old male patient presented with mild cytopenias and massive splenomegaly. Splenectomy showed diffuse red pulp involvement by a pleomorphic medium- to large-cell T-cell lymphoma with a CD2+ CD3+ CD5- CD7- CD4+ CD8+/- CD30- TCRbeta-F1+ immunophenotype, an activated cytotoxic profile, and strong MUM1 expression. The clinical course was marked by disease progression in the bone marrow under treatment and death at 4 months. In contrast with two t(6;14)(p25;q11.2)-positive lymphomas previously reported to be cytotoxic PTCL, NOS with bone marrow and skin involvement, this case was manifested by massive splenomegaly, expanding the clinical spectrum of PTCLs harboring t(6;14)(p25;q11.2) and supporting consideration of this translocation as a marker of biological aggressiveness.
Mots-clé
Chromosomes, Human, Pair 14/genetics, Chromosomes, Human, Pair 6/genetics, Humans, In Situ Hybridization, Fluorescence, Lymphoma, T-Cell, Peripheral/genetics, Lymphoma, T-Cell, Peripheral/pathology, Male, Middle Aged, Splenomegaly/genetics, Splenomegaly/pathology, Translocation, Genetic
Pubmed
Web of science
Création de la notice
08/08/2014 20:40
Dernière modification de la notice
12/12/2023 8:10
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