Skip to main content
SpringerLink
Log in

Splenic Marginal Zone Lymphoma

  • Reference work entry
  • First Online:
Hematopathology

Part of the book series: Encyclopedia of Pathology ((EP))

  • 1184 Accesses

Synonyms

Splenic lymphoma with villous lymphocytes

Definition

The term splenic marginal zone lymphoma (SMZL) was initially proposed by Schmid and Isaacson in 1992 (Schmid et al. 1992) for describing a B-cell lymphoma involving the spleen with a micronodular infiltration pattern and a peculiar morphology suggesting marginal zone origin. The splenic histology and peripheral blood features in these cases was overlapping with those found in the condition termed as “splenic lymphoma with villous lymphocytes” (Matutes et al. 1994); thus both conditions were assumed to represent the same lymphoproliferative disorder and integrated into a single condition, denominated as splenic marginal zone lymphoma.

The use of the term “marginal zone lymphoma” (MZL) has been frequently understood as indicating a close relationship to the other marginal zone–derived tumors, marginal zone lymphoma in mucosa-associated lymphoid tissue (MZL-MALT), and nodal MZL. However, the clinical, immunophenotypic, and...

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Log in via an institution
Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 499.99
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Hardcover Book
USD 549.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References and Further Reading

  • Audouin, J., Le Tourneau, A., Molina, T., Camilleri-Broet, S., Adida, C., Comperat, E., Benattar, L., Delmer, A., Devidas, A., Rio, B., & Diebold, J. (2003). Patterns of bone marrow involvement in 58 patients presenting primary splenic marginal zone lymphoma with or without circulating villous lymphocytes. British Journal of Haematology, 122(3), 404–412.

    Article  PubMed  Google Scholar 

  • Baseggio, L., Traverse-Glehen, A., Petinataud, F., Callet-Bauchu, E., Berger, F., Ffrench, M., Couris, C. M., Thieblemont, C., Morel, D., Coiffier, B., Salles, G., & Felman, P. (2010). CD5 expression identifies a subset of splenic marginal zone lymphomas with higher lymphocytosis: a clinico-pathological, cytogenetic and molecular study of 24 cases. Haematologica 95(4), 604–612.

    Google Scholar 

  • Curiel-Olmo, S., Mondejar, R., Almaraz, C., Mollejo, M., Cereceda, L., Mares, R., Derdak, S., Campos-Martin, Y., Batlle, A., Gonzalez de Villambrosia, S., Gut, M., Blanc, J., Traverse-Glehen, A., Verney, A., Baseggio, L., Camacho, F. I., Wotherspoon, A., Stamatopoulos, K., Xochelli, A., Papadaki, T., Kanellis, G., Ponzoni, M., Garcia-Cosio, M., Vaque, J. P., Beltran, S., Gut, I., Piris, M. A., & Martinez, N. (2017). Splenic diffuse red pulp small B-cell lymphoma displays increased expression of cyclin D3 and recurrent CCND3 mutations. Blood 129(8), 1042–1045.

    Google Scholar 

  • Franco, V., Florena, A. M., & Campesi, G. (1996). Intrasinusoidal bone marrow infiltration: A possible hallmark of splenic lymphoma. Histopathology, 29(6), 571–575.

    Article  CAS  PubMed  Google Scholar 

  • Hamadeh, F., MacNamara, S. P., Aguilera, N. S., Swerdlow, S. H., Cook, J. R. (2014). MYD88 L265P mutation analysis helps define nodal lymphoplasmacytic lymphoma. Modern Pathology.

    Google Scholar 

  • Jallades, L., Baseggio, L., Sujobert, P., Huet, S., Chabane, K., Callet-Bauchu, E., Verney, A., Hayette, S., Desvignes, J. P., Salgado, D., Levy, N., Beroud, C., Felman, P., Berger, F., Magaud, J. P., Genestier, L., Salles, G., & Traverse-Glehen, A. (2017). Exome sequencing identifies recurrent BCOR alterations and the absence of KLF2, TNFAIP3 and MYD88 mutations in splenic diffuse red pulp small B-cell lymphoma. Haematologica 102(10), 1758–1766.

    Google Scholar 

  • Jimenez, C., Sebastian, E., Chillon, M. C., Giraldo, P., Mariano Hernandez, J., Escalante, F., Gonzalez-Lopez, T. J., Aguilera, C., de Coca, A. G., Murillo, I., Alcoceba, M., Balanzategui, A., Sarasquete, M. E., Corral, R., Marin, L. A., Paiva, B., Ocio, E. M., Gutierrez, N. C., Gonzalez, M., San Miguel, J. F., & Garcia-Sanz, R. (2013). MYD88 L265P is a marker highly characteristic of, but not restricted to, Waldenstrom’s macroglobulinemia. Leukemia 27(8), 1722–1728.

    Google Scholar 

  • Kanellis, G., Mollejo, M., Montes-Moreno, S., Rodriguez-Pinilla, S. M., Cigudosa, J. C., Algara, P., Montalban, C., Matutes, E., Wotherspoon, A., & Piris, M. A. (2010). Splenic diffuse red pulp small B-cell lymphoma: revision of a series of cases reveals characteristic clinico-pathological features. Haematologica 95(7), 1122–1129.

    Google Scholar 

  • Kiel, M. J., Velusamy, T., Betz, B. L., Zhao, L., Weigelin, H. G., Chiang, M. Y., Huebner-Chan, D. R., Bailey, N. G., Yang, D. T., Bhagat, G., Miranda, R. N., Bahler, D. W., Medeiros, L. J., Lim, M. S., & Elenitoba-Johnson, K. S. (2012). Whole-genome sequencing identifies recurrent somatic NOTCH2 mutations in splenic marginal zone lymphoma. Journal of Experimental Medicine, 209(9), 1553–1565.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Liu, L., Wang, H., Chen, Y., Rustveld, L., Liu, G., & Du, X. L. (2013). Splenic marginal zone lymphoma: a population-based study on the 2001-2008 incidence and survival in the United States. Leuk Lymphoma 54(7), 1380–1386.

    Google Scholar 

  • Martinez-Lopez, A., Curiel-Olmo, S., Mollejo, M., Cereceda, L., Martinez, N., Montes-Moreno, S., Almaraz, C., Revert, J. B., & Piris, M. A. (2015). MYD88 (L265P) somatic mutation in marginal zone B-cell lymphoma. Am J Surg Pathol 39(5), 644–651.

    Google Scholar 

  • Martinez, N., Almaraz, C., Vaque, J. P., Varela, I., Derdak, S., Beltran, S., Mollejo, M., Campos-Martin, Y., Agueda, L., Rinaldi, A., Kwee, I., Gut, M., Blanc, J., Oscier, D., Strefford, J. C., Martinez-Lopez, J., Salar, A., Sole, F., Rodriguez-Peralto, J. L., Diez-Tascon, C., Garcia, J. F., Fraga, M., Sebastian, E., Alves, J., Menarguez, J., Gonzalez-Carrero, J., Casado, L. F., Bayes, M., Bertoni, F., Gut, I., & Piris, M. A. (2014). Whole-exome sequencing in splenic marginal zone lymphoma reveals mutations in genes involved in marginal zone differentiation. Leukemia, 28(6), 1334–1340.

    Article  CAS  PubMed  Google Scholar 

  • Mateo, M., Mollejo, M., Villuendas, R., Algara, P., Sanchez-Beato, M., Martinez, P., & Piris, M. A. (1999). 7q31-32 allelic loss is a frequent finding in splenic marginal zone lymphoma. American Journal of Pathology, 154(5), 1583–1589.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Matutes, E., Morilla, R., Owusu-Ankomah, K., Houlihan, A., & Catovsky, D. (1994). The immunophenotype of splenic lymphoma with villous lymphocytes and its relevance to the differential diagnosis with other B-cell disorders. Blood, 83(6), 1558–1562.

    Article  CAS  PubMed  Google Scholar 

  • Matutes, E., Oscier, D., Montalban, C., Berger, F., Callet-Bauchu, E., Dogan, A., Felman, P., Franco, V., Iannitto, E., Mollejo, M., Papadaki, T., Remstein, E. D., Salar, A., Sole, F., Stamatopoulos, K., Thieblemont, C., Traverse-Glehen, A., Wotherspoon, A., Coiffier, B., & Piris, M. A. (2008). Splenic marginal zone lymphoma proposals for a revision of diagnostic, staging and therapeutic criteria. Leukemia, 22(3), 487–495.

    Article  CAS  PubMed  Google Scholar 

  • Mollejo, M., Lloret, E., Menarguez, J., Piris, M. A., & Isaacson, P. G. (1997). Lymph node involvement by splenic marginal zone lymphoma: Morphological and immunohistochemical features. American Journal of Surgical Pathology, 21(7), 772–780.

    Article  CAS  PubMed  Google Scholar 

  • Montalban, C., Abraira, V., Arcaini, L., Domingo-Domenech, E., Guisado-Vasco, P., Iannitto, E., Mollejo, M., Matutes, E., Ferreri, A. J., Salar, A., Rattotti, S., Carpaneto, A., Perez, R., Bello, J. L., Hernandez, M., Caballero, D., Carbonell, F., Piris, M. A., & Splenic Marginal Zone Lymphoma Study, G. (2014). Simplification of risk stratification for splenic marginal zone lymphoma: a point-based score for practical use. Leuk Lymphoma 55(4), 929–931.

    Google Scholar 

  • Piva, R., Deaglio, S., Fama, R., Buonincontri, R., Scarfo, I., Bruscaggin, A., Mereu, E., Serra, S., Spina, V., Brusa, D., Garaffo, G., Monti, S., Dal Bo, M., Marasca, R., Arcaini, L., Neri, A., Gattei, V., Paulli, M., Tiacci, E., Bertoni, F., Pileri, S. A., Foa, R., Inghirami, G., Gaidano, G., Rossi, D. (2014). The Kruppel-like factor 2 transcription factor gene is recurrently mutated in splenic marginal zone lymphoma. Leukemia.

    Google Scholar 

  • Ponzoni, M., Kanellis, G., Pouliou, E., Baliakas, P., Scarfo, L., Ferreri, A. J., Doglioni, C., Bikos, V., Dagklis, A., Anagnostopoulos, A., Ghia, P., Stamatopoulos, K., & Papadaki, T. (2012). Bone marrow histopathology in the diagnostic evaluation of splenic marginal-zone and splenic diffuse red pulp small B-cell lymphoma: A reliable substitute for spleen histopathology? American Journal of Surgical Pathology, 36(11), 1609–1618.

    Article  PubMed  Google Scholar 

  • Rossi, D., Trifonov, V., Fangazio, M., Bruscaggin, A., Rasi, S., Spina, V., Monti, S., Vaisitti, T., Arruga, F., Fama, R., Ciardullo, C., Greco, M., Cresta, S., Piranda, D., Holmes, A., Fabbri, G., Messina, M., Rinaldi, A., Wang, J., Agostinelli, C., Piccaluga, P. P., Lucioni, M., Tabbo, F., Serra, R., Franceschetti, S., Deambrogi, C., Daniele, G., Gattei, V., Marasca, R., Facchetti, F., Arcaini, L., Inghirami, G., Bertoni, F., Pileri, S. A., Deaglio, S., Foa, R., Dalla-Favera, R., Pasqualucci, L., Rabadan, R., & Gaidano, G. (2012). The coding genome of splenic marginal zone lymphoma: Activation of NOTCH2 and other pathways regulating marginal zone development. Journal of Experimental Medicine, 209(9), 1537–1551.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Schmid, C., Kirkham, N., Diss, T., & Isaacson, P. G. (1992). Splenic marginal zone cell lymphoma. American Journal of Surgical Pathology, 16(5), 455–466.

    Article  CAS  PubMed  Google Scholar 

  • Troen, G., Warsame, A., & Delabie, J. (2013). CD79B and MYD88 mutations in splenic marginal zone lymphoma. ISRN Oncology, 2013, 252318.

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  • Xochelli, A., Bikos, V., Polychronidou, E., Galigalidou, C., Agathangelidis, A., Charlotte, F., Moschonas, P., Davis, Z., Colombo, M., Roumelioti, M., Sutton, L. A., Groenen, P., van den Brand, M., Boudjoghra, M., Algara, P., Traverse-Glehen, A., Ferrer, A., Stalika, E., Karypidou, M., Kanellis, G., Kalpadakis, C., Mollejo, M., Pangalis, G., Vlamos, P., Amini, R. M., Pospisilova, S., Gonzalez, D., Ponzoni, M., Anagnostopoulos, A., Giudicelli, V., Lefranc, M. P., Espinet, B., Panagiotidis, P., Piris, M. A., Du, M. Q., Rosenquist, R., Papadaki, T., Belessi, C., Ferrarini, M., Oscier, D., Tzovaras, D., Ghia, P., Davi, F., Hadzidimitriou, A., & Stamatopoulos, K. (2019). Disease-biased and shared characteristics of the immunoglobulin gene repertoires in marginal zone B cell lymphoproliferations. Journal of Pathology,247(4): 416–421.

    Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Servicio de AnatomíaPatológica, Hospital Virgen de la Salud, Toledo, Spain

    M. Mollejo

  2. Pathology Service, Hospital Universitario Fundación Jiménez Díaz, Madrid, Spain

    Miguel A. Piris

Authors
  1. M. Mollejo
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Miguel A. Piris
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Miguel A. Piris .

Editor information

Editors and Affiliations

  1. Department of Pathology, Paris Descartes University, University of Paris, Necker-Enfants Malades Hospital, AP-HP, Paris, France

    Thierry J. Molina

Rights and permissions

Reprints and permissions

Copyright information

© 2020 Springer Nature Switzerland AG

About this entry

Check for updates. Verify currency and authenticity via CrossMark

Cite this entry

Mollejo, M., Piris, M.A. (2020). Splenic Marginal Zone Lymphoma. In: Molina, T.J. (eds) Hematopathology. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-319-95309-0_1938

Download citation

  • DOI: https://doi.org/10.1007/978-3-319-95309-0_1938

  • Published:

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-95308-3

  • Online ISBN: 978-3-319-95309-0

  • eBook Packages: MedicineReference Module Medicine

Publish with us

Policies and ethics

Search

Navigation