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Clinical and biological features of B‐cell neoplasms with CDK6 translocations: an association with a subgroup of splenic marginal zone lymphomas displaying frequent CD5 expression, prolymphocytic cells, and TP53 abnormalities

Baptiste Gailllard 1 Pascale Cornillet‐lefebvre 1 Quoc‐hung Le 1 Karim Maloum 2 Mélanie Pannetier 3 Carinne Lecoq‐lafon 1 Béatrice Grange 4 Ludovic Jondreville 5 Lucienne Michaux 6 Nathalie Nadal 7 Antoine Ittel 8 Isabelle Luquet 9 Stéphanie Struski 9 Christine Lefebvre 10 Jean‐baptiste Gaillard 11 Marina Lafage‐pochitaloff 12 Estelle Balducci 13 Dominique Penther 14 Carole Barin 15 Marie Agnès Collonge‐rame 16 Mélanie Jimenez‐poquet 17 Steven Richebourg 18 Sabine Defasque 19 Isabelle Radford‐weiss 20 Audrey Bidet 21 Santos Susin 5 Florence Nguyen‐khac 2 Elise Chapiro 2 Pierre Lemaire 22
Abstract : A translocation involving the cyclin‐dependent kinase 6 (CDK6) gene [t(CDK6)] is a rare but recurrent abnormality in B‐cell neoplasms. To further characterise this aberration, we studied 57 cases; the largest series reported to date. Fluorescence in situ hybridisation analysis confirmed the involvement of CDK6 in all cases, including t(2;7)(p11;q21) immunoglobulin kappa locus (IGK)/CDK6 (n = 51), t(7;14)(q21;q32) CDK6/immunoglobulin heavy locus (IGH) (n = 2) and the previously undescribed t(7;14)(q21;q11) CDK6/T‐cell receptor alpha locus (TRA)/T‐cell receptor delta locus (TRD) (n = 4). In total, 10 patients were diagnosed with chronic lymphocytic leukaemia, monoclonal B‐cell lymphocytosis or small lymphocytic lymphoma, and 47 had small B‐cell lymphoma (SmBL) including 36 cases of marginal zone lymphoma (MZL; 34 splenic MZLs, one nodal MZL and one bronchus‐associated lymphoid tissue lymphoma). In all, 18 of the 26 cytologically reviewed cases of MZL (69%) had an atypical aspect with prolymphocytic cells. Among the 47 patients with MZL/SmBL, CD5 expression was found in 26 (55%) and the tumour protein p53 (TP53) deletion in 22 (47%). The TP53 gene was mutated in 10/30 (33%); the 7q deletion was detected in only one case, and no Notch receptor 2 (NOTCH2) mutations were found. Immunoglobulin heavy‐chain variable‐region (IGHV) locus sequencing revealed that none harboured an IGHV1‐02*04 gene. Overall survival was 82% at 10 years and not influenced by TP53 aberration. Our present findings suggest that most t(CDK6)+ neoplasms correspond to a particular subgroup of indolent marginal zone B‐cell lymphomas with distinctive features.
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Baptiste Gailllard, Pascale Cornillet‐lefebvre, Quoc‐hung Le, Karim Maloum, Mélanie Pannetier, et al.. Clinical and biological features of B‐cell neoplasms with CDK6 translocations: an association with a subgroup of splenic marginal zone lymphomas displaying frequent CD5 expression, prolymphocytic cells, and TP53 abnormalities. British Journal of Haematology, Wiley, 2020, ⟨10.1111/bjh.17141⟩. ⟨hal-03099358⟩

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