Lymphoma - B cell neoplasms - Splenic lymphoma with villous lymphocytes

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Lymphoma - B cell neoplasms

B cell lymphoma subtypes

Splenic lymphoma with villous lymphocytes

Reviewer: Nikhil Sangle, M.D., University of Utah and ARUP Laboratories (see Reviewers page)
Revised: 20 March 2011, last major update March 2011
Copyright: (c) 2001-2011, PathologyOutlines.com, Inc.

Definition
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● Rare disorder, usually considered the leukemic variant of splenic marginal zone B cell lymphoma

Clinical features
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● Resembles hairy cell leukemia clinically
● Mean age 72 years, usually males
● Marked splenomegaly
● Prolonged clinical course

Laboratory
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● 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
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● Splenectomy if severe cytopenia
● May enter remission after treatment for concurrent hepatitis C with interferon

Micro description
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● 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
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● CD19, CD22, CD24, intense surface immunoglobulin, CD11c (50%), CD25 (25%), CD5 (19%); variable TRAP

Molecular
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● 7q21-22 deletion (40%), trisomy 3 (17%)

Differential diagnosis
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● Hairy cell leukemia

End of Lymphoma - B cell neoplasms > B cell lymphoma subtypes > Splenic lymphoma with villous lymphocytes

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