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Clinical manifestations, pathologic features, and diagnosis of extranodal (MALT) and nodal marginal zone lymphomas

Arnold S Freedman, MD
Jon C Aster, MD
Section Editor
Andrew Lister, MD, FRCP, FRCPath, FRCR
Deputy Editor
Rebecca F Connor, MD


The non-Hodgkin lymphoma subtype of marginal zone lymphoma includes three distinct diseases that have been historically classified together because they appear to arise from post-germinal center marginal zone B cells and share a similar immunophenotype: positive for B cell markers CD19, CD20, and CD22, and negative for CD5, CD10, and usually CD23 [1,2].

The three diseases in this category that are recognized in the 2016 World Health Organization classification of lymphoid neoplasms are:

Extranodal marginal zone B cell lymphoma of mucosa associated lymphoid tissue (MALT) type (MALT-type lymphoma, MALT lymphoma)

Nodal marginal zone B cell lymphoma

Splenic marginal zone B cell lymphoma (± villous lymphocytes)

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Literature review current through: Sep 2017. | This topic last updated: Apr 26, 2016.
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