Definition
Nodal marginal zone lymphoma (NMZL) is a rare type of B-cell lymphoma. It is defined as a nodal B-cell neoplasm that resembles lymph nodes involved by splenic marginal zone lymphoma (SMZL) or extranodal marginal zone lymphoma (EMZL) but without evidence of splenic or extranodal disease.
Clinical Features
Incidence
NMZL is a rare type of B-cell lymphoma, accounting for less than 2% of lymphoid neoplasms. However, the incidence of NMZL appears to be increasing (Olszewski and Castillo 2012).
Age
The median age of presentation is around 60 years, but there is a wide variation with an age range from 13 to 92 years. Especially in younger patients, the possibility of the pediatric form of marginal zone lymphoma should be considered.
Sex
Males and females are affected at approximately equal frequency.
Site
By definition, the disease presents in the lymph nodes with the absence of extranodal or splenic disease. Most...
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References and Further Reading
Olszewski, A. J., & Castillo, J. J. (2012). Survival of patients with marginal zone lymphoma: Analysis of the Surveillance, Epidemiology, and End Results database. Cancer, 119, 629–638.
Salama, M. E., Lossos, I. S., Warnke, R. A., & Natkunam, Y. (2009). Immunoarchitectural patterns in nodal marginal zone B-cell lymphoma: A study of 51 cases. American Journal of Clinical Pathology, 132, 39–49.
Spina, V., Khiabanian, H., Messina, M., Monti, S., Cascione, L., Bruscaggin, A., Spaccarotella, E., Holmes, A. B., Arcaini, L., Lucioni, M., Tabbo, F., Zairis, S., Diop, F., Cerri, M., Chiaretti, S., Marasca, R., Ponzoni, M., Deaglio, S., Ramponi, A., Tiacci, E., Pasqualucci, L., Paulli, M., Falini, B., Inghirami, G., Bertoni, F., Foa, R., Rabadan, R., Gaidano, G., & Rossi, D. (2016). The genetics of nodal marginal zone lymphoma. Blood, 128, 1362–1373.
Van den Brand, M., & Van Krieken, J. H. J. M. (2013). Recognizing nodal marginal zone lymphoma: Recent advances and pitfalls. A systematic review. Haematologica, 98, 1003–1013.
Van den Brand, M., Rijntjes, J., Hebeda, K. M., Menting, L., Bregitha, C. V., Stevens, W. B. C., Van der Velden, W. J. F. M., Tops, B. B. J., Van Krieken, J. H. J. M., & Groenen, P. J. T. A. (2017). Recurrent mutations in genes involved in NF-kappaB signaling in nodal marginal zone lymphoma. Histopathology, 70, 174–184.
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van den Brand, M. (2019). Nodal Marginal Zone Lymphoma. In: van Krieken, J. (eds) Encyclopedia of Pathology. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-319-28845-1_3869-1
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DOI: https://doi.org/10.1007/978-3-319-28845-1_3869-1
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