MCL


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  • Clinical

    • median age 60
    • 3-10% of non-Hodgkins
    • site: nodes, spleen, marrow, blood
    • lymphomatoid papulosis in colon

  • Morphology

    • architecture: diffuse, vaguely nodular or mantle zone pattern
    • monomorphic centrocyte-like cells: smaller cells, cleaved nuclei, inconspicuous nucleoli
    • lacks centroblasts

  • Variants

    • blastoid: aggressive, lymphoblast-like, high mitotic rate (usually 20-30 per 10 hpf)
    • pleomorphic: aggressive, pleomorphic
    • small cell: resembles CLL
    • marginal zone-like: abundant pale cytoplasm

  • Stains

    • positive: CD5, BCL2, cyclinD1 (BCL1), SOX11
    • negative: CD10, BCL6

  • Molecular

    • t(11;14)
    • cyclinD1 (BCL1, CCND1) on chr 11 with IGH on chr 14
    • classical MCL: unmutated IGH, worse prognosis
    • mutated IGH: splenomegaly, lymphocytosis, better prognosis

  • Cyclin D1 negative MCL

    • SOX11 expression
    • negative for cyclin D1 and t(11;14)
    • positive for cyclin D2 or cyclin D3 and other MCL markers
    • can have t(2;12), cyclin D2 kappa fusion
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