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

Details

Serval ID
serval:BIB_19B29CF3637B
Type
Article: article from journal or magazin.
Publication sub-type
Case report (case report): feedback on an observation with a short commentary.
Collection
Publications
Institution
Title
Peripheral T-cell lymphoma with t(6;14)(p25;q11.2) translocation presenting with massive splenomegaly.
Journal
Virchows Archiv
Author(s)
Somja J., Bisig B., Bonnet C., Herens C., Siebert R., de Leval L.
ISSN
1432-2307 (Electronic)
ISSN-L
0945-6317
Publication state
Published
Issued date
2014
Peer-reviewed
Oui
Volume
464
Number
6
Pages
735-741
Language
english
Notes
Publication types: Case Reports ; Journal ArticlePublication Status: ppublish PDF: Case Report
Abstract
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.
Keywords
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
Create date
08/08/2014 20:40
Last modification date
20/08/2019 13:50
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