Article: article from journal or magazin.
Case report (case report): feedback on an observation with a short commentary.
Peripheral T-cell lymphoma with t(6;14)(p25;q11.2) translocation presenting with massive splenomegaly.
Publication types: Case Reports ; Journal ArticlePublication Status: ppublish PDF: Case Report
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.
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
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