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Blood, 15 February 2008, Vol. 111, No. 4, pp. 2253-2260. Prepublished online as a Blood First Edition Paper on November 27, 2007; DOI 10.1182/blood-2007-07-098848.
Submitted July 2, 2007
Service d'Anatomie Pathologique Centre Hospitalier Lyon Sud, Hospices Civils de Lyon, Lyon, France * Corresponding author; email: pascale.felman{at}chu-lyon.fr.
The presence of circulating villous lymphocytes (VL) in lymphoma patients usually points to splenic marginal zone B cell lymphoma (SMZL), even if they can be found occasionally in other small B-cell lymphomas. However those cells are variably described and detailed cytological characterization is often lacking. We identified lymphoma cases with numerous basophilic VL among the large group of splenic lymphoma with villous lymphocytes, and for further delineation, 37 cases with this particular cytology were analyzed. Patients, predominantly older males, presented with moderate lymphocytosis and splenomegaly without pancytopenia. The monoclonal B cells expressed IgM+D, IgM+G, IgM or IgG, as well as CD76 and CD11c, frequently CD103 and rarely CD123. Spleen sections were peculiar with atrophic white pulp and a monomorphic diffuse lymphoma infiltration in a congested red pulp. Bone marrow infiltration was interstitial and intrasinusoidal without extensive fibrosis. Cytogenetic analysis showed a frequent absence of clonal aberrations (68%). Most cases (79%) were IgH mutated with an over-representation of VH3 and VH4 gene families. These results, as well as the clinical evolution, show that those lymphoma cases represent a homogeneous group, distinct from SMZL and reminiscent of HCL-Variant, perhaps corresponding to a separate lymphoma entity.
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