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The heterogeneity of follicular lymphomas: from early development to transformation

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Abstract

Follicular lymphoma (FL) is a lymphoma composed of germinal center B cells, i.e., centroblasts and centrocytes, that almost always show at least a focal follicular growth pattern. Most cases have a characteristic CD5-, CD10+, BCL6+, and BCL2+ immunophenotype, and 85 % of cases exhibit the hallmark translocation t(14;18)(q32;q21) involving BCL2 and IGH. Although the typical clinicopathological findings of FL are well recognized, cases with unusual clinical, morphologic, immunophenotypic, and genetic features may pose problems in diagnosis and nomenclature. In the slide workshop organized by the European Association for Haematopathology (EAHP) and the Society for Hematopathology (SH) held in Istanbul, Turkey, unusual variants of FL were discussed based on the submitted cases, including early lesions, localized extranodal presentation, uncommon immunophenotype, rare genetic alterations, diffuse variant, and marginal zone differentiation. Interesting features such as blastoid morphology and unusual progression forms were presented, aiming to understand the genetic basis of transformation. In this report, novel findings and diagnostic challenges emerging from the submitted cases will be highlighted, and new terminologies for some of these lesions are proposed.

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Acknowledgments

We thank all participants of the workshop for submitting their cases. The permission to use their images in this review is also gratefully acknowledged.

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Correspondence to Luc Xerri.

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Xerri, L., Dirnhofer, S., Quintanilla-Martinez, L. et al. The heterogeneity of follicular lymphomas: from early development to transformation. Virchows Arch 468, 127–139 (2016). https://doi.org/10.1007/s00428-015-1864-y

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