Lymphoma and plasma cell neoplasms
B cell lymphoma subtypes
Splenic marginal zone B cell lymphoma

Author: Nikhil Sangle, M.D. (see Authors page)

Revised: 3 April 2017, last major update March 2011

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PubMed Search: splenic marginal zone B cell lymphoma

Cite this page: Splenic marginal zone B cell lymphoma. PathologyOutlines.com website. http://pathologyoutlines.com/topic/lymphomasplenicmzl.html. Accessed October 22nd, 2017.
Definition / general
  • Rare low grade B cell lymphoma of middle age 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
  • 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 Waldenström macroglobulinemia; are similar otherwise to nonplasmacytic 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
Gross description
  • Multiple small, grayish white nodules
Gross images

Images hosted on other servers:

Markedly enlarged spleen with nodular cut surface

Microscopic (histologic) description
  • 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
Microscopic (histologic) images

Images hosted on other servers:

Various images

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

Positive stains
Negative stains
Molecular / cytogenetics description
  • 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