Lymphoma and plasma cell neoplasms
B cell lymphoma subtypes
Lymphoplasmacytic lymphoma

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

Revised: 3 April 2017, last major update February 2011

Copyright: (c) 2001-2017, PathologyOutlines.com, Inc.

PubMed Search: lymphoplasmacytic lymphoma

Cite this page: Lymphoplasmacytic lymphoma. PathologyOutlines.com website. http://pathologyoutlines.com/topic/lymphomalpl.html. Accessed June 23rd, 2017.
Definition / general
  • Plasmacytic lymphoma that lacks diagnostic criteria for other B cell lymphomas
Terminology
  • Also called plasmacytoid lymphoma, immunocytoma
Clinical features
  • 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 Waldenström 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 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 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 phenomena and cold urticaria
Laboratory
  • 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 Waldenström macroglobulinemia (above)
Prognostic factors
  • May have poorer prognosis than other small B cell lymphomas if advanced age, peripheral blood cytopenias and high β2 microglobulin levels (Am J Surg Pathol 2001;25:742)
Case reports
Treatment
  • Cannot cure
  • Use plasmapheresis for hyperviscosity and hemolysis
Microscopic (histologic) description
  • 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
Microscopic (histologic) images

Images hosted on other servers:

Various images

With peliosis hepatis: H&E and BCL10

Bone marrow

Positive stains
  • 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
Molecular / cytogenetics description
Differential diagnosis