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Lymphoma - B cell neoplasms

B cell lymphoma subtypes

Lymphoplasmacytic lymphoma


Reviewer: Nikhil Sangle, M.D., University of Utah and ARUP Laboratories (see Reviewers page)
Revised: 24 February 2011, last major update February 2011
Copyright: (c) 2001-2011, PathologyOutlines.com, Inc.

Definition
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● Plasmacytic lymphoma that lacks diagnostic criteria for other B cell lymphomas

Terminology
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● Also called plasmacytoid lymphoma, immunocytoma

Clinical features
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● Rare (2% of hematopoietic neoplasms) malignancy of older patients (50-69 years) with involvement of bone marrow, lymph node, spleen and liver
● May affect nerve roots, meninges and rarely brain or small intestine (Arch Pathol Lab Med 2001;125:677)
● No masses causing bony erosions as seen with multiple myeloma
● Does not fulfill the criteria for any other B-cell lymphoma with plasmacytic differentiation
● Most patients have monoclonal IgM and Waldenstrom’s macroglobulinemia with hyperviscosity symptoms
● 10% have autoimmune hemolysis due to cold agglutinins (IgM antibodies that bind at < 37C)
● Median survival is 5 years, 40% survive 10 years or more or die of unrelated causes
● May transform to diffuse large B cell lymphoma with Reed-Sternberg like cells or immunoblasts

Waldenström's macroglobulinemia: clinical syndrome defined by serum IgM monoclonal gammopathy, monoclonal Ig light chains (Bence-Jones protein) in urine, hyperviscosity syndrome and B cell neoplasm involving marrow, including lymphoplasmacytic lymphoma (Am J Surg Pathol 2003;27:1104), B-cell CLL/SLL, marginal zone B cell lymphoma (MALT type), myeloma

Diagnostic criteria for Waldenström's recommended by Second International Workshop:
● IgM monoclonal gammopathy of any concentration
● Bone marrow infiltration by small lymphocytes showing plasmacytoid or plasma cell differentiation
● Intertrabecular pattern of bone marrow infiltration
● Immunophenotype is surface IgM+, CD19+, CD20+, CD22+, CD25+, CD27+, FMC7+, CD5 variable, CD10-, CD23-, CD103-, CD108-

Hyperviscosity syndrome: due to IgM’s large size and number; symptoms are visual impairment (distended and tortuous retinal veins with hemorrhage and exudates), neurologic symptoms due to sluggish blood flow, bleeding due to IgM binding of clotting factors and cryoglobulinemia causing Raynaud’s phenomena and cold urticaria

Laboratory
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● Moderate to severe normochromic anemia with marked rouleaux formation (like partially stacked coins)
● 30% have leukemia composed of lymphocytes and lymphoplasmacytoid cells
● Also features of Waldenstrom’s macroglobulinemia (above)

Prognostic features
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● May have poorer prognosis than other small B cell lymphomas if advanced age, peripheral blood cytopenias and high beta2 microglobulin levels (Am J Surg Pathol 2001;25:742)

Case reports
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● With peliosis hepatis (Arch Pathol Lab Med 2004;128:1283)

Treatment
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● Cannot cure
● Use plasmapheresis for hyperviscosity and hemolysis

Micro description
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● Partial or complete nodal effacement by small B cells with plasmacytic differentiation in 3 architectural patterns (a) open sinuses with small follicles and hemosiderosis, (b) hyperplastic follicles, (c) diffuse plasma cells, lymphocytes and "plymphyocytes”
● By definition, excludes other small B cell lymphomas
● Often has reactive mast cells, epithelioid histiocytes, cytoplasmic (Russell) and nuclear (Dutcher) PAS positive inclusions; inclusions are cytoplasmic immunoglobulin

Bone Marrow: focal or diffuse involvement by small B cells with plasmacytic differentiation and cytoplasmic (Russell) and nuclear (Dutcher) PAS positive inclusions
● Inclusions are cytoplasmic immunoglobulin and are nonspecific
● Diffuse involvement is associated with decrease in normal hematopoietic cells
● Often has reactive mast cells, epithelioid histiocytes, mature plasma cells

Micro images
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Various images


With peliosis hepatis: H&E and bcl-10


Bone marrow

Positive stains
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● CD19, CD79a, surface and cytoplasmic immunoglobulin (IgM, IgD)
● Also variable CD20, CD22, CD38, variable CD43, CD138 and strong cytoplasmic immunoglobulin in plasma cell component (monoclonal kappa or lambda, not both)

Negative stains
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● CD5 (usually), CD10, CD23, bcl2, CD103

Molecular
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● t(9;14)(p13;q32): PAX5 and IgH in 0% (Hum Pathol 2004;35:447) to 50%, may be more common in nodal tumors
● PAX5 encodes B cell specific activator protein (transcription factor)
● No t(1;14)(p22;q32) or t(11;18)(q21;q21) of MALT lymphoma (Mod Pathol 2004;17:1150)
● Aneuploidy is associated with polymorphous subtype and poor prognosis (Am J Clin Pathol 2001;116:543)

Differential diagnosis
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Myeloma: CD20 negative, strong CD138+; rarely has serum hyperviscosity
● Serum hyperviscosity also seen in CLL/SLL (CD5 positive), marginal zone B cell lymphoma-MALT type (Am J Clin Pathol 2001;116:683)

End of Lymphoma - B cell neoplasms > B cell lymphoma subtypes > Lymphoplasmacytic lymphoma


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