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The many faces of small B cell lymphomas with plasmacytic differentiation and the contribution of MYD88 testing

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Abstract

Plasmacytic differentiation may occur in almost all small B cell lymphomas (SBLs), although it varies from being uniformly present (as in lymphoplasmacytic lymphoma (LPL)) to very uncommon (as in mantle cell lymphomas (MCLs)). The discovery of MYD88 L265P mutations in the vast majority of LPLs has had a major impact on the study of these lymphomas. Review of the cases contributed to the 2014 European Association for Haematopathology/Society for Hematopathology slide workshop illustrated how mutational testing has helped refine the diagnostic criteria for LPL, emphasizing the importance of identifying a clonal monotonous lymphoplasmacytic population and highlighting how LPL can still be diagnosed with extensive nodal architectural effacement, very subtle plasmacytic differentiation, follicular colonization, or uncommon phenotypes such as CD5 or CD10 expression. MYD88 L265P mutations were found in 11/11 LPL cases versus only 2 of 28 other SBLs included in its differential diagnosis. Mutational testing also helped to exclude other cases that would have been considered LPL in the past. The workshop also highlighted how plasmacytic differentiation can occur in chronic lymphocytic leukemia/small lymphocytic lymphoma, follicular lymphoma, SOX11 negative MCL, and particularly in marginal zone lymphomas, all of which can cause diagnostic confusion with LPL. The cases also highlighted the difficulty in distinguishing lymphomas with marked plasmacytic differentiation from plasma cell neoplasms. Some SBLs with plasmacytic differentiation can be associated with amyloid, other immunoglobulin deposition, or crystal-storing histiocytosis, which may obscure the underlying neoplasm. Finally, although generally indolent, LPL may transform, with the workshop cases suggesting a role for TP53 abnormalities.

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Acknowledgments

The authors are very grateful to the participants who contributed a large number of very interesting and informative cases to this workshop and for the use of their cases and images in this review.

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Authors and Affiliations

  1. Division of Hematopathology, Department of Pathology, University of Pittsburgh School of Medicine, Room G-335, 200 Lothrop Street, Pittsburgh, PA, 15213, USA

    Steven H. Swerdlow

  2. Department of Pathology, Ankara University School of Medicine, Sihhiye, Ankara, Turkey

    Isinsu Kuzu

  3. Hematopathology Service, Departments of Pathology and Laboratory Medicine, Memorial Sloan Kettering Cancer Center, New York, NY, USA

    Ahmet Dogan

  4. Institute of Pathology, University Hospital Basel, Basel, Switzerland

    Stephan Dirnhofer

  5. Queen Elizabeth Hospital, Hong Kong, SAR, China

    John K. C. Chan

  6. Department of Laboratory Medicine, Division of Pathology, Karolinska Institut and Karolinska University Hospital Huddinge, Stockholm, Sweden

    Birgitta Sander

  7. Department of Clinical Pathology, Robert-Bosch-Hospital and Dr. Margarete Fischer-Bosch Institute of Clinical Pharmacology, Stuttgart, Germany

    German Ott

  8. Department of Bio-Pathology, Institut Paoli-Calmettes and Aix-Marseille University, Marseille, France

    Luc Xerri

  9. Institute of Pathology and Neuropathology, Eberhard Karls University of Tübingen and Comprehensive Cancer Center, University Hospital Tübingen, Tübingen, Germany

    Leticia Quintanilla-Martinez

  10. Hospital Clinic, University of Barcelona, Barcelona, Spain

    Elias Campo

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Swerdlow, S.H., Kuzu, I., Dogan, A. et al. The many faces of small B cell lymphomas with plasmacytic differentiation and the contribution of MYD88 testing. Virchows Arch 468, 259–275 (2016). https://doi.org/10.1007/s00428-015-1858-9

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