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Cutaneous Marginal Zone Lymphoma : Immunophenotype

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Cutaneous Marginal Zone Lymphoma (CMZL) - Immunohistochemistry : The immunohistochemical profile of primary CMZL is similar to other MALT lymphomas. Briefly, the neoplastic marginal zone cells are positive for CD20, PAX5, CD79a, bcl-2, and CD43 (aberrant expression, indicative of neoplastic phenotype). They are negative for CD5, CD10, CD23, bcl-6 and cyclinD1. Rare cases may be CD5+. The background shows scattered, unremarkable CD3+ T-cells.

The plasma cells express CD138, IRF-4/MUM1 and CD79a but are negative for CD20.

The associated reactive lymphoid follicles have expanded follicular dendritic cell networks which can be highlighted by CD21, CD23, and/or CD35 immunostains. The tumor cells also show immunoglobulin light chain restriction (kappa or lambda) and immunoglobulin heavy chain gene rearrangements. CMZL arising in immunocompromised setting (e.g. post-transplant) are EBV-positive.

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