Article (Scientific journals)
Peripheral T-cell lymphoma with t(6;14)(p25;q11.2) translocation presenting with massive splenomegaly.
SOMJA, Joan; Bisig, B.; de Leval, L. et al.
2014In Virchows Archiv
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Keywords :
Peripheral T-cell lymphoma; Translocation; Splenomegaly; IRF4/MUM1; T-cell receptor alpha; Cytotoxic
Abstract :
[en] 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.
Disciplines :
Hematology
Author, co-author :
SOMJA, Joan ;  Centre Hospitalier Universitaire de Liège - CHU > Anatomie pathologique
Bisig, B.
de Leval, L.
Bonnet, Christophe ;  Université de Liège - ULiège > GIGA-R : Hématologie
HERENS, Christian ;  Centre Hospitalier Universitaire de Liège - CHU > Génétique
Siebert, R.
Language :
English
Title :
Peripheral T-cell lymphoma with t(6;14)(p25;q11.2) translocation presenting with massive splenomegaly.
Publication date :
2014
Journal title :
Virchows Archiv
ISSN :
0945-6317
eISSN :
1432-2307
Publisher :
Springer International, Berlin, Germany
Peer reviewed :
Peer Reviewed verified by ORBi
Available on ORBi :
since 24 April 2014

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