Surgical Pathology Criteria

Splenic Marginal Zone B Cell Lymphoma

Differential Diagnosis


Extranodal vs. Nodal vs. Splenic Marginal Zone Lymphomas

  Extranodal Nodal Splenic
7q21-32 loss rare rare 40%
trisomy 3 60% rare 17%
t(11;18) 25-50% rare 0%


Small B Cell Lymphomas
  SLL/CLL Mantle Marginal Zone Lymphoplasmacytic Follicular
CD23 85% 2% 8% 0-30% on immunohistology but up to 60% weak positive on flow 0-25%
CD5 80% 80% 0% 5% 0%
bcl1 2% 85% 0% 0% 0%
CD10 0% 2% 2% 3% 85%
CD43 80% 85% 35% 10-30% 7%
bcl2 95% 95% 65-90% >50% 90%


Splenic Marginal Zone Lymphoma SLL/CLL
Nodular pattern with prominent marginal zone Diffuse effacement
Polymorphous cell population Uniform small round lymphocytes
May have residual germinal centers Proliferation centers frequent
May have circulating villous lymphocytes No villi on circulating cells
CD23 8% CD23 85%
CD5 negative CD5 80%
CD43 2% CD43 80%


Nodal, Extranodal and Splenic Marginal Zone Lymphoma Lymphoplasmacytic Lymphoma
Enlarged marginal zone Diffuse effacement
Pale monocytoid cells No monocytoid population


Nodal, Extranodal and Splenic Marginal Zone Lymphoma Mantle Cell Lymphoma
Enlarged marginal and mantle zones Enlarged mantle zone frequent
Polymorphous population Uniform small lymphocytes
bcl1 negative bcl1 85%
CD25 negative CD25 30%
CD5 negative CD5 80%
CD43 35% (Splenic 2%) CD43 85%


Splenic Marginal Zone Lymphoma Follicular Lymphoma
Polymorphous population Uniform population of atypical small lymphocytes
Centers of nodules may contain bcl2 negative residual germinal centers Centers of nodules composed of bcl2 positive small atypical lymphocytes
Plasmacytoid differentiation frequent Plasmacytoid differentiation rare
CD10 2% CD10 85%
No peripheral adenopathy May have peripheral adenopathy
May have circulating villous lymphocytes May have circulating prolymphocytes


Nodal, Extranodal and Splenic Marginal Zone Lymphoma Mast Cell Disease
B lineage markers positive B lineage markers negative
Toluidine blue, Giemsa stains negative Toluidine blue, Giemsa stains positive
CD117, tryptase immuno stains negative CD117, tryptase immuno stains positive
Pale marginal zone cells may simulate mast cells


Nodal, Extranodal and Splenic Marginal Zone Lymphoma Marginal Zone Hyperplasia
Monocytoid cells may be prominent No monocytoid population
Light chain monotypia variably demonstrable No monotypia
Clonal Ig gene rearrangement No clonal rearrangement
Coexpression of CD43 by B cells 35% No CD43 coexpression
Sheets of B cells Distinct B and T cell zones


Nodal, Extranodal and Splenic Marginal Zone Lymphoma with Large Cell Transformation Sporadic Diffuse Large B Cell Lymphoma
Prior or concurrent specimen with typical marginal zone lymphoma No evidence of typical marginal zone lymphoma
CD5 negative CD5 10%
CD10 2% CD10 25-50%
bcl6 negative bcl6 60%
Both may involve the GI tract and other mucosal sites


Plasmacytoma / Myeloma Lymphoplasmacytic Lymphoma and Nodal, Extranodal and Splenic Marginal Zone Lymphomas
Plasma cells may be pleomorphic or plasmablastic Plasma cell component usually not markedly atypical
Uniform plasma cell morphology Plasma cells mixed with small lymphocytes
IgG or IgA M component most common IgM or IgG M component most common
CD20 often negative CD20 80%
Uniformly CD138 positive Scattered CD138 positive cells
Often CD56+, CD19-, CD45- CD56-, CD19+, CD45+

Splenic Marginal Zone Lymphoma Hairy Cell Leukemia
DBA44 negative DBA44 positive
Tartrate resistant acid phosphatase negative Tartrate resistant acid phosphatase positive
Biphasic cell sizes Uniform cells with small nuclei and abundant cytoplasm
Villi if present are polar Hairy projections circumferential
Nodular pattern of white pulp involvement White pulp atrophic
Peripheral nodes only rarely involved Peripheral nodes may be involved
Both typically involve the spleen and may have circulating cells with cytoplasmic processes/villi

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