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Splenic lymphoma with circulating villous lymphocytes
Splenic lymphoma with villous lymphocytes
Splenic marginal zone B-cell lymphoma
Splenic marginal zone lymphoma, NOS
Very rare disease characterized by lymphoid infiltrate in splenic white pulp growing in nodular pattern replacing pre-existing follicles with variable degree of red pulp infiltration; small lymphocytes predominant in central areas, medium-sized cells present in periphery. 66% present with circulating villous lymphocytes.
Splenomegaly is seen in almost all patients, commonly without lymphadenopathy. The bone marrow is frequently positive. Nodal and extranodal involvment are rare.
The lack of CD5 expression is helpful in the discrimination between SMZL and chronic lymphocytic leukemia/small lymphocytic lymphoma, and the lack of CD10 expression argues against follicular lymphoma. Mantle cell lymphoma is excluded due to the lack of CD5 and cyclin-D1 expression.
Definitive Diagnostic Methods
Acute loss of chromosome 7q31-32
Immunoglobulin heavy and light chain rearrangement