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(American Journal of Pathology. 2003;162:681-689.)
© 2003 American Society for Investigative Pathology

Regular Articles

Splenic Marginal Zone Lymphoma with Villous Lymphocytes Shows On-Going Immunoglobulin Gene Mutations

Anne Tierens*, Jan Delabie*, Agnieszka Malecka*, Junbai Wang{dagger}, Alicja Gruszka-Westwood{ddagger}, Daniel Catovsky{ddagger} and Estella Matutes{ddagger}

From the Departments of Pathology* and Tumor Biology,{dagger} The Norwegian Cancer Institute and Radiumhospital, University of Oslo, Oslo, Norway; and Academic Haematology and Cytogenetics,{ddagger} The Royal Marsden Hospital, London, United Kingdom

Splenic marginal zone lymphoma (also splenic lymphoma with villous lymphocytes) is a B-cell non-Hodgkin’s lymphoma with a characteristic morphology and phenotype. We studied the pattern of somatic hypermutation of the rearranged immunoglobulin heavy chain genes on 23 cases and have correlated these data with survival as well as immunophenotypic and genetic characteristics of the cases. Two-thirds of the cases show immunoglobulin gene mutations, half of which show evidence of antigen selection, whereas one-third of the cases show no significant mutations. On-going mutation, a feature characteristic of follicular lymphoma, was demonstrated in all six cases randomly selected for this analysis, including one case with a low number of mutations (<2%). No statistical significant correlation was found between immunoglobulin mutation status and clinical, immunophenotypic, or genetic characteristics. Our results demonstrate that on-going somatic hypermutation is a prominent feature of splenic marginal zone lymphoma with circulating villous lymphocytes. On-going somatic hypermutation has previously been demonstrated in extra-nodal and nodal marginal zone lymphoma. Our results indicate that marginal zone lymphomas at different anatomical localizations may derive from a similar B-cell subset.




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