Abstract
IntroductionDiscordant lymphomas are rare entities characterized by the simultaneous presence of two distinct types of lymphomas in different anatomic sites. We describe a very rare case of simultaneous occurrence of splenic mantle cell lymphoma and marginal zone lymphoma involving the bone marrow and peripheral blood.Case presentationWe report the case of a 60-year-old asymptomatic Caucasian woman in whom discordant lymphomas were discovered when a slight lymphocytosis and a conspicuous splenomegaly were observed. The different morphological, immunophenotypical and immunohistochemical features found in the different pathologic samples obtained from peripheral blood, bone marrow and spleen sections made it possible to differentiate two types of non-Hodgkin B-cell lymphomas: a mantle cell lymphoma infiltrating the spleen and a marginal zone lymphoma involving both the bone marrow and peripheral blood. Since a similar IgH gene rearrangement was found both in the bone marrow and in the spleen, the hypothesis of a common origin, followed by a different clonal selection of the neoplastic lymphocytes may be taken into consideration.ConclusionOur case emphasizes the usefulness of investigating simultaneous specimens from different anatomic sites from the same patient and the relevant diagnostic role of splenectomy.
Highlights
Discordant lymphomas are rare entities characterized by the simultaneous presence of two distinct types of lymphomas in different anatomic sites
We describe a patient with mantle cell lymphoma (MCL) concomitant with a marginal zone lymphoma
The bone marrow biopsy specimens showed a global cellularity of 50% with nodular-interstitial infiltration by CD20+ and bcl2+ lymphocytes, which accounted for 15% of cellularity and was negative for CD5, CD23, bcl6, cyclin-D1 and DBA44 (Figure 2)
Summary
The association of two distinct B-cell non-Hodgkin lymphomas in the same patient and involving different anatomical locations is a rare phenomenon. The patient was referred to the Division of Hematology for further observation and underwent bone marrow evaluation (morphology by myeloaspirate specimens, trephine biopsy, flow cytometry, molecular biology assays and karyotype). The bone marrow biopsy specimens showed a global cellularity of 50% with nodular-interstitial infiltration by CD20+ and bcl2+ lymphocytes, which accounted for 15% of cellularity and was negative for CD5, CD23, bcl, cyclin-D1 and DBA44 (Figure 2). Flow cytometry of bone marrow blood showed 16% lymphocytes which were positive for CD19, CD20, CD22, CD103 and surface K light chain, and negative for CD5, CD23, CD10, CD11c, CD25, and surface l light chain (Figure 3). DNA extracted from paraffin-embedded spleen specimens (EZ1 Advanced, Qiagen) was subjected to PCR assays, which showed a clonal IgH rearrangement, the same as found in the bone marrow (Figures 5 and 6). Therapy with the cyclophosphamide, hydroxydaunorubicin (Adriamycin), Oncovin (vincristine) and prednisone - rituximab (CHOP-R) protocol was started and, after six courses and restaging by computed tomography and bone marrow and peripheral blood investigation, showed that complete remission was achieved
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