Lymphoma - B cell neoplasms - Marginal zone lymphoma

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Lymphoma - B cell neoplasms

B cell lymphoma subtypes

Marginal zone lymphoma - nodal

Reviewer: Nikhil Sangle, M.D., University of Utah and ARUP Laboratories (see Reviewers page)
Revised: 7 February 2012, last major update March 2011
Copyright: (c) 2001-2011, PathologyOutlines.com, Inc.

Definition
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● Uncommon low grade lesion, associated with Sjogren's syndrome, that resembles MALT lymphoma with monocytoid, centrocyte-like cells
● For diagnosis, must rule out evidence of extranodal or splenic disease

Clinical features
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● Uncommon
● Similar incidence in men and women
● Mean age 61 years (range 26-92 years)
● Usually associated with Sjogren’s syndrome
● Associated with Hepatitis C in some studies (Br J Haematol 2007;136:301)
● Usually localized lymphadenopathy (59% stage I-II at diagnosis)
● Marrow and blood involvement are rare
● Usually indolent; 5 year survival is 57%-79%; may be longer than extranodal marginal cell lymphoma
● May be preceded by marginal zone hyperplasia with clear cells (Am J Clin Pathol 2001;116:550)

● MALT subtype: perisinusoidal, perivascular infiltration of monocytoid / centrocytic cells and residual germinal centers with well-preserved mantle cuff; negative for IgD, cyclin D1, CD5; 44% had masses in salivary gland, soft tissue, skin or breast suggestive of extranodal lymphoma (Am J Surg Pathol 1999;23:59)

● Splenic subtype: polymorphic infiltrate surrounding residual germinal centers, no or attenuated mantle cuff; bcl2+ (83%); IgD+ (100%), CD5-, CD23-, cyclinD1-; resembles follicular lymphoma, but residual germinal centers were bcl2-, no t(14;18); no splenomegaly (Am J Surg Pathol 1999;23:59)

Features in children/young adults
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● 67% of marginal zone B cell lymphomas in children / young adults (up to age 29 years) are nodal, usually involving head and neck nodes
● 80% male, usually no underlying disease
● Excellent prognosis with low risk of recurrence
● Have partial to total effacement of lymph nodes with obliteration of sinuses by atypical cells with predominantly interfollicular distribution and marked expansion of marginal zones; disruption of residual follicles in 2/3 resembles progressive transformation of germinal centers; cells are polymorphic, small to medium size, some with moderate cytoplasm and round nuclei and others with scant cytoplasm and irregular nuclei; frequent plasma cells and larger transformed cells, few/rare blast cells (Am J Surg Pathol 2003;27:522)

Prognostic factors
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● Follicular lymphoma international prognostic index (FLIPI) can be used to predict prognosis (Br J Haematology 2007;136:301)

Case reports
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● Biclonal disease (Arch Pathol Lab Med 2000;124:1816)

Micro description
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● Partial/total effacement of architecture, interfollicular infiltrate of monocytoid, centrocyte-like B cells that are 2-3x larger than small lymphocytes
● Tumor cells surround and infiltrate follicles, have moderately abundant pale cytoplasm, round/irregular nuclei with clumped chromatin
● Similar morphology to MALT lymphoma or splenic marginal zone B cell lymphoma
● Also has benign follicular centers
● 30% have neoplastic plasma cells
● Presence of follicular dendritic meshwork remnant suggestive of colonized follicles favors diagnosis of marginal zone lymphoma

Micro images
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Axillary node: Fig A/B-H&E; Fig C-CD20

Positive stains
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● CD19, CD79a, bcl2
● Also CD22, IgM, IgD
● Variable CD11c
● A classification with immunohistochemical and molecular heterogeneity (Am J Surg Pathol 2003;27:762)

Negative stains
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● CD5, CD10, CD23, CD25, bcl10, cyclin D1
● Variable CD43 and bcl6

Molecular
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● Clonal rearrangements of IgH and light chains
● Trisomy 18 and 3; 1q21 or 1q34 abnormalities

Differential diagnosis
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● Follicular lymphoma: CD10+, bcl6+, t(14,18)
● Lymphoid hyperplasia: has reactive follicles and normal appearing cells, no destructive changes, no lymphoepithelial features
● SLL/CLL: CD5+, CD23+
● Mantle cell lymphoma: CD5+, cyclin D1+
● Hairy cell leukemia: CD103+, CD11c+, CD25+
● Mastocytosis: tryptase+
● Lymphoplasmacytic lymphoma
● Lymphocyte predominance Hodgkin’s lymphoma
● Acute monocytic leukemia
● Toxoplasmic lymphadenitis

End of Lymphoma - B cell neoplasms > B cell lymphoma subtypes > Marginal zone lymphoma - nodal

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