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Follicular lymphoma without t(14;18) and with BCL-6 rearrangement: a lymphoma subtype with distinct pathological, molecular and clinical characteristics

Leukemia volume 16, pages 2309–2317 (2002)Cite this article

Abstract

Translocations involving the BCL-6 gene are frequently observed in diffuse large B cell lymphoma, but have rarely been reported in follicular lymphoma (FL). We studied a distinct cohort of FLs with a 3q27/BCL-6 gene rearrangement, but lacking the t(14;18) translocation. In 13/15 cases, translocations involved the 3q27 and the 14q32 regions. All cases displayed a marked follicular growth pattern and, in some instances, a monocytoid component. Tumor cells were CD5− CD20+ CD23− CD43− BCL-6+, and in the main CD10 negative (n = 10, 71%) and BCL-2 negative (n = 11, 78%). When compared to 20 typical t(14;18)+ FLs, the presence of large follicles (P = 0.01) and a CD10−/BCL-2− phenotype were more frequently observed (P = 0.001) in our cohort. Clonal mutations arising in the BCL-6 first intron were observed in 5/7 cases with evidence of intraclonal heterogeneity, consistent with a germinal center origin. No significant difference was found in comparison to t(14;18)+ FL regarding age, sex, performance status, bone marrow involvement or overall survival. However, in the 3q27+ FL group, a stage III/IV disease and a bulky mass were less frequently observed. This study indicates that 3q27+ FL without t(14;18) translocation have peculiar clinico-pathologic features and may correspond to a rare and distinct subtype of lymphoma originating from the germinal center.

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Article 12 December 2019

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Acknowledgements

This work was supported by the Comité Départemental de Seine Maritime de la Ligue Nationale Contre le Cancer. The authors thank C Haioun for providing clinical data, C Lamarche for her technical help and S Sahota for his critical review of the manuscript.

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Authors and Affiliations

  1. Department of Haematology and EMI 9906-IRFMP No. 23, Centre Henri Becquerel, Rouen, France

    F Jardin, G Buchonnet, N Contentin, S Leprêtre, P Lenain, A Stamatoullas & H Tilly

  2. Department of Pathology, Centre Henri Becquerel, Rouen, France

    JM Picquenot & C Duval

  3. Molecular Biology Laboratory and EMI 9906-IRFMP No. 23, Centre Henri Becquerel, Rouen, France

    F Parmentier & C Bastard

  4. Department of Pathology and EA 2348, Hôpital Henri Mondor, AP-HP, Créteil, France

    P Gaulard

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Jardin, F., Gaulard, P., Buchonnet, G. et al. Follicular lymphoma without t(14;18) and with BCL-6 rearrangement: a lymphoma subtype with distinct pathological, molecular and clinical characteristics. Leukemia 16, 2309–2317 (2002). https://doi.org/10.1038/sj.leu.2402707

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