Nodal Marginal Zone Lymphoma
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.
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).
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.
Males and females are affected at approximately equal frequency.
By definition, the disease presents in the lymph nodes with the absence of extranodal or splenic disease. Most...
References and Further Reading
- 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.CrossRefGoogle Scholar
- 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.CrossRefGoogle Scholar