Lymphoma & related disorders

Mature B cell neoplasms

Small B cell lymphomas with lymphoplasmacytic differentiation / marginal zone lymphomas

Marginal zone-general

Last author update: 1 March 2011
Last staff update: 16 June 2021

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PubMed Search: marginal zone B cell lymphoma

Nikhil Sangle, M.D.
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Cite this page: Sangle N. Marginal zone-general. website. Accessed September 21st, 2023.
Definition / general
  • 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
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
  • Plasmacytoid cells may be negative for B cell markers but are strongly immunoreactive for kappa or lambda, and carry a serum paraprotein (M-component) in peripheral blood
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