Lymphoma & related disorders
Mature B cell neoplasms
Small B cell lymphomas with lymphoplasmacytic differentiation / marginal zone lymphomas
Splenic lymphoma with villous lymphocytes


Topic Completed: 1 March 2011

Minor changes: 16 March 2020

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PubMed Search: splenic lymphoma with villous lymphocytes


Nikhil Sangle, M.D.
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Cite this page: Sangle N. Splenic lymphoma with villous lymphocytes. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/lymphomaspleniclymphomavillous.html. Accessed September 24th, 2020.
Definition / general
  • Rare disorder, usually considered the leukemic variant of splenic marginal zone B cell lymphoma
Clinical features
  • Resembles hairy cell leukemia clinically
  • Mean age 72 years, usually males
  • Marked splenomegaly
  • Prolonged clinical course
Laboratory
  • Peripheral blood demonstrates lymphocytosis (mild / moderate), usually WBC < 25 billion/L; anemia and thrombocytopenia in 50%
  • Lymphocytes have short villous projections, usually with polar orientation; may have distinct nucleoli; may have plasmacytoid lymphocytes
  • 50% have IgM gammopathy
Treatment
  • Splenectomy if severe cytopenia
  • May enter remission after treatment for concurrent hepatitis C with interferon
Microscopic (histologic) description
  • Almost all cases have marrow involvement with variable patterns
  • Small or scattered large lymphocytes with abundant cytoplasm and prominent nucleoli; no / rare mitotic figures
  • Often prominent plasma cells
Positive stains
Molecular / cytogenetics description
  • 7q21-22 deletion (40%), trisomy 3 (17%)
Differential diagnosis
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