LW35: PARTIAL NODAL INVOLVEMENT BY NODAL MARGINAL ZONE LYMPHOMA (VERSUS NODAL MARGINAL ZONE LYMPHOMA IN-SITU)

Darshan Roy1, Rebecca King1 and Adam Bagg1 1. University of Pennsylvania, Department of Pathology and Laboratory Medicine, Philadelphia, USA darshan.roy@uphs.upenn.edu

Clinical history: A 44-year-old female patient presented with a history of fever, chills, and night sweats. Clinically, she had neck lymphadenopathy. A CT scan shows retroperitoneal lymphadenopathy and splenomegaly. CBC: WBC 3.5 THO/ul, HgB 11.2 g/dl, Plt: 215 THO/ul. Automated differential: Neutrophils: 55.8%, Lymphocytes: 34.6%, Monocytes: 6.6%, Eosinophils: 1.5%, Basophils: 1.5%. Details of biopsy: Cervical, Level 2, biopsy. The specimen was received as 2 potential lymph nodes measuring 4 x 2 x 1 cm overall. Serial sectioning revealed a yellow-tan smooth surface. A portion of the specimen was sent for flow cytometry, another part placed in B5 fixative, remainder submitted in toto in formalin. Immunohistochemistry/immunophenotype: Immunohistochemistry performed with adequate controls demonstrates the mostly normal topographic distribution of immunophenotypically-normal CD20+ CD79a+ Bcells in the secondary follicles, with CD3+ CD5+ CD43+ T-cells predominantly populating the interfollicular zones. There is also a mild expansion of B-cells in the interfollicular zones. The germinal center cells in the secondary follicles are CD10+ BCL6+ Ki-67+ BCL2-, with Ki-67 highlighting their polarization. The more cytologically homogeneous nodular structures (potentially mimicking primary follicles) are also populated by CD20+ CD79a+ B cells; however, these cells clearly co-express CD43 and BCL2, but are essentially negative for CD5, CD10, BCL6, cyclin D1 and Ki-67. CD21 and CD23 highlight the expanded follicular dendritic meshworks; although mostly intact, in areas corresponding to the CD43+ B cell expansions, the follicular dendritic meshworks appear to be disrupted. Flow cytometry performed on this lymph node shows a mixture of phenotypically normal T cells and polytypic B cells. No evidence of lymphoma was identified. Genetic/molecular analysis: Immunoglobulin Heavy Chain Gene Rearrangement by PCR Positive (monoclonal), Framework 2: 272 bp peak, Framework 3:106 bp (doublet) peak. Proposed diagnosis Lymph node with partial involvement by nodal marginal zone lymphoma (versus nodal marginal zone lymphoma in-situ) Interesting feature(s) of the submitted case This case was originally evaluated by surgical pathology and initially thought to be a reactive node. Morphologically the lymph node has patent sinuses with an expansion of both primary and secondary follicles; this impression was bolstered by the normal flow cytometric analysis. The case was submitted to hematopathology to bless this diagnostic consideration. Upon closer examination the nodules ("primary follicles") have cytologic atypia with nuclear irregularity and moderately abundant cytoplasm. Additional evaluation shows these nodules to be dominated by CD43+ B-cells, suggesting that these so-called primary follicles are actually extensively colonized by a B-cell lymphoma. Thus, even though much of the lymph node appears histologically reactive (supported by the flow cytometric data), there is indeed histologic, morphologic, immunohistochemical, and molecular evidence of (partial involvement) by a small B-cell lymphoma that is best classified as a nodal marginal zone lymphoma; a widely disseminated splenic marginal zone lymphoma seems less likely. Since the lymphoma was initially not obviously apparent, it might be considered an in-situ nodal marginal zone lymphoma. This is not widely recognized, and may be akin to recent descriptions of insitu follicular and mantle cell lymphomas.