Lymphoma and plasma cell neoplasms
B cell lymphoma subtypes
Splenic lymphoma with villous lymphocytes

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

Revised: 3 April 2017, last major update March 2011

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PubMed Search: splenic lymphoma with villous lymphocytes

Cite this page: Splenic lymphoma with villous lymphocytes. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/lymphomaspleniclymphomavillous.html. Accessed April 26th, 2017.
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
Micro 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