Reference : Peripheral T-cell lymphoma with t(6;14)(p25;q11.2) translocation presenting with mass...
Scientific journals : Article
Human health sciences : Hematology
Peripheral T-cell lymphoma with t(6;14)(p25;q11.2) translocation presenting with massive splenomegaly.
SOMJA, Joan mailto [Centre Hospitalier Universitaire de Liège - CHU > > Anatomie pathologique >]
Bisig, B. []
de Leval, L. []
Bonnet, Christophe mailto [Université de Liège - ULiège > > GIGA-R : Hématologie >]
HERENS, Christian mailto [Centre Hospitalier Universitaire de Liège - CHU > > Génétique >]
Siebert, R. []
Virchows Archiv
Springer International
Yes (verified by ORBi)
[en] Peripheral T-cell lymphoma ; Translocation ; Splenomegaly ; IRF4/MUM1 ; T-cell receptor alpha ; Cytotoxic
[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.

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