Virchows Archiv

, Volume 468, Issue 3, pp 259–275 | Cite as

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

  • Steven H. Swerdlow
  • Isinsu Kuzu
  • Ahmet Dogan
  • Stephan Dirnhofer
  • John K. C. Chan
  • Birgitta Sander
  • German Ott
  • Luc Xerri
  • Leticia Quintanilla-Martinez
  • Elias Campo
Review and Perspectives
First Online:
Received:
Accepted:

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.

Keywords

Lymphoplasmacytic lymphoma MYD88 Marginal zone lymphoma Mantle cell lymphoma Chronic lymphocytic leukemia Follicular lymphoma Plasmacytic differentiation 
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Notes

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.

Compliance with ethical standards

Conflict of interest

The authors declare that they have no conflict of interest.

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Copyright information

© Springer-Verlag Berlin Heidelberg 2015

Authors and Affiliations

  • Steven H. Swerdlow
    • 1
  • Isinsu Kuzu
    • 2
  • Ahmet Dogan
    • 3
  • Stephan Dirnhofer
    • 4
  • John K. C. Chan
    • 5
  • Birgitta Sander
    • 6
  • German Ott
    • 7
  • Luc Xerri
    • 8
  • Leticia Quintanilla-Martinez
    • 9
  • Elias Campo
    • 10
  1. 1.Division of Hematopathology, Department of PathologyUniversity of Pittsburgh School of MedicinePittsburghUSA
  2. 2.Department of PathologyAnkara University School of MedicineSihhiyeTurkey
  3. 3.Hematopathology Service, Departments of Pathology and Laboratory Medicine, Memorial Sloan Kettering Cancer CenterNew YorkUSA
  4. 4.Institute of PathologyUniversity Hospital BaselBaselSwitzerland
  5. 5.Queen Elizabeth HospitalHong KongChina
  6. 6.Department of Laboratory Medicine, Division of PathologyKarolinska Institut and Karolinska University Hospital HuddingeStockholmSweden
  7. 7.Department of Clinical PathologyRobert-Bosch-Hospital and Dr. Margarete Fischer-Bosch Institute of Clinical PharmacologyStuttgartGermany
  8. 8.Department of Bio-PathologyInstitut Paoli-Calmettes and Aix-Marseille UniversityMarseilleFrance
  9. 9.Institute of Pathology and NeuropathologyEberhard Karls University of Tübingen and Comprehensive Cancer Center, University Hospital TübingenTübingenGermany
  10. 10.Hospital ClinicUniversity of BarcelonaBarcelonaSpain

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