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Lymphoma - B cell neoplasms

B cell lymphoma subtypes

Splenic marginal zone B cell lymphoma

Reviewer: Nikhil Sangle, M.D., University of Utah and ARUP Laboratories (see Reviewers page)
Revised: 20 March 2011, last major update March 2011
Copyright: (c) 2001-2011, PathologyOutlines.com, Inc.

Definition
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● Rare low grade B cell lymphoma of middle aged and elderly patients (median age 69 years, no gender preference)
● Marginal zone: Light zone surrounding splenic follicles which contains post-follicular memory B cells derived after stimulation of recirculating cells from T cell dependent antigen

Clinical features
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● Usually disseminated (as opposed to MALT lymphoma) with splenomegaly and left upper quadrant pain, interstitial and focal paratrabecular marrow involvement in 75% causing anemia, involvement of peripheral blood, liver and often splenic hilar lymph nodes, but no peripheral nodal involvement unless it transforms
● Usually indolent but 13% transform, possibly related to 7q deletion (Am J Surg Pathol 2001;25:1268)
● Note: cases with plasmacytic differentiation often present with monoclonal serum disorders and autoimmune disorders, including hemolytic anemia; may have Waldenstrom’s macroglobulinemia; are similar otherwise to non-plasmacytic cases (Am J Surg Pathol 2000;24:1581)
● Peripheral smear shows cells with scant cytoplasm and cleaved nucleus; confirm neoplastic with flow cytometry

Case reports
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● 22 year old woman (Arch Pathol Lab Med 2002;126:214)
● 52 year old woman with spontaneous partial regression associated with regression of acquired C1 esterase inhibitor deficiency (J Clin Pathol 2002;55:789)
● 70 year old woman with blastic transformation (Arch Pathol Lab Med 2000;124:748)
● 73 year old man with coexisting Gaucher’s disease with transformation to diffuse large B cell lymphoma (Arch Pathol Lab Med 2003;127:e242)

Gross description
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● Multiple small, grey-white nodules

Gross images
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Markedly enlarged spleen with nodular cut surface

Micro description
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● Infiltration of small atypical lymphocytes in mantle zone and medium lymphocytes with pale cytoplasm and oval clear nucleus in marginal zone, leading to mixed mantle-zone and marginal-zone involvement pattern
● Variable follicular colonization but definite increase in white pulp
● Cells are centrocyte-like, monocytoid (easily recognized with imprints) or lymphoplasmacytic
● <20% immunoblasts; involves red pulp also
● Intrasinusoidal infiltration of bone marrow is relatively specific finding
● Bone marrow intrasinusoidal pattern is fairly specific, highlighted by CD20; may also have focal paratrabecular and nonparatrabecular pattern, and benign germinal centers

Micro images
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Prominent white pulp with marginal zone expansion; marginal zone cells have moderate cytoplasm and intermediate nuclei


Various images

Positive stains
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● CD19, CD20, CD22, CD45RA, CD79a, bcl2
● Tumor cells are IgM+ and IgD (dim), but there is no IgD positive mantle area

Negative stains
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● CD3, CD5, bcl-1/cyclin D1, CD10, CD23
● Also CD43, CD25

Molecular
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● Clonal rearrangements of IgH and IgL are common; often gains in chromosomes X, 3 and 18 and losses in 6q and 7q31-32 (Mod Pathol 2003;16:1210)

Differential diagnosis
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● Lymphoplasmacytic lymphoma: no pale corona surrounding reactive or colonized germinal centers and no monocytoid B cells in marginal zone
● B-CLL: CD5+
● Persistent polyclonal B cell lymphocytosis: Mod Pathol 2004;17:1087

End of Lymphoma - B cell neoplasms > B cell lymphoma subtypes > Splenic marginal zone B cell lymphoma

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