Clinical manifestations, pathologic features, and diagnosis of splenic marginal zone lymphoma
- Arnold S Freedman, MD
Arnold S Freedman, MD
- Section Editor — Lymphoproliferative Disorders
- Professor of Medicine
- Harvard Medical School
- Jon C Aster, MD
Jon C Aster, MD
- Professor of Pathology
- Harvard Medical School
The non-Hodgkin lymphoma subtype marginal zone lymphoma encompasses 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 [1-3].
The three diseases in this category that are recognized in the 2008 WHO classification system of lymphoid neoplasms include:
●Splenic marginal zone B cell lymphoma (± villous lymphocytes)
●Extranodal marginal zone B cell lymphoma of mucosa associated lymphoid tissue (MALT) type (MALT-type lymphoma, MALT lymphoma) 
●Nodal marginal zone B cell lymphoma
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- CLINICAL FEATURES
- PATHOLOGIC FEATURES
- - Peripheral blood
- - Spleen
- - Lymph nodes
- - Bone marrow
- Genetic features
- DIFFERENTIAL DIAGNOSIS
- Follicular lymphoma
- Mantle cell lymphoma
- Chronic lymphocytic leukemia
- Hairy cell leukemia
- Lymphoplasmacytic lymphoma (Waldenström macroglobulinemia)
- MALT lymphoma
- Splenic B cell lymphoma/leukemia, unclassifiable
- Monoclonal B cell lymphocytosis