title: Peripheral T-cell lymphoma with t(6;14)(p25;q11.2) translocation presenting with massive splenomegaly.
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.
authors.affiliation: Department of Pathology, B35, University of Liege, CHU Sart Tilman, Liege, Belgium, Joan.Somja@ulg.ac.be.
authors.forename: Joan
authors.initials: J
authors.lastname: Somja
authors.forename: Bettina
authors.initials: B
authors.lastname: Bisig
authors.forename: Christophe
authors.initials: C
authors.lastname: Bonnet
authors.forename: Christian
authors.initials: C
authors.lastname: Herens
authors.forename: Reiner
authors.initials: R
authors.lastname: Siebert
authors.forename: Laurence
authors.initials: L
authors.lastname: de Leval
mesh_terms: D002883:Chromosomes, Human, Pair 14 / Q000235:genetics*; D002896:Chromosomes, Human, Pair 6 / Q000235:genetics*; D006801:Humans; D017404:In Situ Hybridization, Fluorescence; D016411:Lymphoma, T-Cell, Peripheral / Q000235:genetics* / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D013163:Splenomegaly / Q000235:genetics* / Q000473:pathology; D014178:Translocation, Genetic