Encyclopedia of Pathology

Living Edition
| Editors: J.H.J.M. van Krieken

Lymphoplasmacytic Lymphoma

  • German Ott
Living reference work entry
DOI: https://doi.org/10.1007/978-3-319-28845-1_3870-1


Lymphoplasmacytic lymphoma (LPL) is defined as a B-cell lymphoma consisting of a mixture of lymphocytes, plasmacytic lymphocytes, and monoclonal plasma cells that does not conform to any other entity of small B-cell lymphoma with plasmacytic differentiation (Swerdlow et al. 2017). LPL typically manifests in the bone marrow and spleen and less frequently in lymph nodes and other organs. Waldenström Macroglobulinemia (WM) is defined as a malignant lymphoma with the morphology of LPL involving the bone marrow and that is associated with monoclonal IgM paraproteinemia; vice versa, an IgM monoclonal paraprotein can be detected in the majority of LPL patients. Identification of the LPL hallmark mutation MYD88 L265P has greatly facilitated and added in the specification of the diagnosis.

Incidence and Epidemiology

LPL is a rare disease representing approximately 2% of non-Hodgkin lymphomas. Patients usually present at a median age of roughly 70 years, and the male gender...

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References and Further Reading

  1. Kristinsson, S. Y., Koshiol, J., Goldin, L. R., Björkholm, M., Turesson, I., Gridley, G., … Landgren, O. (2009). Genetics- and immune-related factors in the pathogenesis of lymphoplasmacytic lymphoma/Waldenström’s macroglobulinemia. Clinical Lymphoma and Myeloma, 9(1), 23–26.  https://doi.org/10.3816/CLM.2009.n.004.CrossRefGoogle Scholar
  2. Lin, P., Mansoor, A., Bueso-Ramos, C., Hao, S., Lai, R., & Medeiros, L. J. (2003). Diffuse large B-cell lymphoma occurring in patients with lymphoplasmacytic lymphoma/Waldenstrom macroglobulinemia. Clinicopathologic features of 12 cases. American Journal of Clinical Pathology, 120(2), 246–253.  https://doi.org/10.1309/R01V-XG46-MFCD-VNHL.CrossRefPubMedGoogle Scholar
  3. Schmidt, J., Federmann, B., Schindler, N., Steinhilber, J., Bonzheim, I., Fend, F., & Quintanilla-Martinez, L. (2015). MYD88 L265P and CXCR4 mutations in lymphoplasmacytic lymphoma identify cases with high disease activity. British Journal of Haematology, 169(6), 795–803.  https://doi.org/10.1111/bjh.13361.CrossRefPubMedGoogle Scholar
  4. Sohani, A. R., & Zukerberg, L. R. (2014). Small B-cell lymphomas of the spleen: How to tell them apart. Journal of Hematopathology, 7(3), 109–121.  https://doi.org/10.1007/s12308-014-0208-1.CrossRefGoogle Scholar
  5. Sohani, A. R., Rodig, S. J., & Harris, N. L. (2017). Lymphoplasmacytic lymphoma and waldenström macroglobulinemia. In E. S. Jaffe, D. A. Arber, E. Campo, N. L. Harris, & L. Quintanilla-Fend (Eds.), Hematopathology (2nd, p. 285). Saunders/Elsevier, Philadelphia.Google Scholar
  6. Staiger, A. M., Ott, M. M., Parmentier, S., Rosenwald, A., Ott, G., Horn, H., & Griese, E.-U. (2015). Allele-specific PCR is a powerful tool for the detection of the MYD88 L265P mutation in diffuse large B cell lymphoma and decalcified bone marrow samples. British Journal of Haematology, 171(1), 145–148.  https://doi.org/10.1111/bjh.13369.CrossRefPubMedGoogle Scholar
  7. Swerdlow, S. H., Kuzu, I., Dogan, A., Dirnhofer, S., Chan, J. K. C., Sander, B., et al. (2016). The many faces of small B cell lymphomas with plasmacytic differentiation and the contribution of MYD88 testing. Virchows Archiv: An International Journal of Pathology, 468(3), 259–275.  https://doi.org/10.1007/s00428-015-1858-9.CrossRefGoogle Scholar
  8. Swerdlow, S. H., Campo, E., Harris, N. L., Jaffe, E. S., Pileri, S. A., Stein, H., & Thiele, J. (Eds.). (2017). WHO classification of tumours of haematopoietic and lymphoid tissues (World Health Organization classification of tumours, Revised 4th ed.). Lyon: International Agency for Research on Cancer.Google Scholar
  9. Treon, S. P., Xu, L., Yang, G., Zhou, Y., Liu, X., Cao, Y., … Hunter, Z. R. (2012). MYD88 L265P somatic mutation in Waldenstrom’s macroglobulinemia. The New England Journal of Medicine, 367(9), 826–833.  https://doi.org/10.1056/NEJMoa1200710.CrossRefGoogle Scholar

Copyright information

© Springer International Publishing AG 2018

Authors and Affiliations

  1. 1.Department of Clinical PathologyRobert-Bosch-KrankenhausStuttgartGermany