Lymphoma & related disorders
Mature B cell neoplasms
Small B cell lymphomas with lymphoplasmacytic differentiation / marginal zone lymphomas
Marginal zone-general
Author: Nikhil Sangle, M.D.
Last author update: 1 March 2011
Last staff update: 16 June 2021
Copyright: 2001-2023, PathologyOutlines.com, Inc.
PubMed Search: marginal zone B cell lymphoma
Table of Contents
Definition / general | Etiology | Clinical features | Microscopic (histologic) description | StainsCite this page: Sangle N. Marginal zone-general. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/lymphomamzlgeneral.html. Accessed September 21st, 2023.
Definition / general
- Three types of lymphomas with overlapping characteristics: MALT lymphoma, nodal marginal zone lymphoma and splenic marginal zone lymphoma
- All feature a perifollicular proliferation of centrocyte or monocyte-like, irregular small B cells, mixed with larger centroblast or immunoblast-like cells; may have plasmacytoid features
Etiology
- Marginal zone lymphomas are thought to derive from cells in the nodal or splenic marginal zone, perhaps from B memory cells that are post-germinal center and IgM+, IgD-, based on a low sequence homology between their IgH variable genes and germline genes
Clinical features
- All usually have bone marrow involvement (Am J Clin Path 2002;117:698)
- Extranodal marginal zone lymphomas in children and young adults are similar to MALT lymphoma in older patients (Am J Surg Pathol 2003;27:522)
Microscopic (histologic) description
- Perifollicular proliferation of centrocyte or monocyte-like, irregular small B cells, mixed with larger centroblast or immunoblast-like cells; may have plasmacytoid features
- The clonal B cells infiltrate the marginal zone of reactive follicles, and extend into the interfollicular region
- In epithelial lined tissues, the clonal B cells infiltrate the epithelial cells to form lymphoepithelial lesions
- Cells resemble monocytoid B cells in lymph node sinuses in toxoplasmosis, cat-scratch disease, AIDS, infectious mononucleosis, autoimmune disorders and other reactive disorders
