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Case 68 - Discussion

Hematopathology Case

 

 

CD20 CD20

CD79a CD3
CD15

CD30

EMA EMA
 
CD23  

Nodular Lymphocyte Predominant Hodgkin Lymphoma (NLPHL)

  • Peak incidence in fourth decade, male preponderance, M:F=2.4:1

  • Typically at early-stage as isolated lymphadenopathy, often involve cervical and axillary nodes

  • Extremely rare marrow involvement, 2.5%

  • Histopathology:

    • Often complete obliteration of nodal architecture

    • Macronodules with loose FDC meshworks, rarely diffuse pattern

    • Scattered tumor cells in the large nodules, “moth-eaten” appearance

    • LH cells: large, complex lobation, popcorn cells; nucleoli medium-sized, generally basophilic, and smaller than those of classic HRS cells; relatively sparse cytoplasm

    • Predominant small lymphocytes in the nodules

    • Groups of epithelioid cells surrounding the nodules in some cases

    • Rarely diffuse pattern (D-LPHL): with cytologic characteristics of NLPHL but lacking evidence of a nodular growth pattern

  • Immunophenotype:

    • Positive: CD45, CD20, BCL6, AID, BSAP, OCT2, BOB1, PU.1, MUM-1, T-bet, GCET1, HGAL/GCET2, IgD, J chain

    • Negative: CD15, CD30, BCL2, CD10, CD138, EBV

    • Background CD4+ T cells: c-Maf+, CD57+, PD-1+, CXCL13+, with T-cell rosettes around LP cells; low ratio of TIA-1+ to CD57+

    • Most cases with reactive CD4+CD8+ cells in 10-38% of T cells

  • Associated with PTGC, preceding or following NLPHL

  • T/HRBCL, most common of lymphoma develops after NLPHL

  • Transformation to DLBCL, 3-10%; not transform to classic HL

  • Excellent outcome, overall event-free survival 90%

  • Differential diagnosis:

    • T-cell rich large B-cell lymphoma: similar immunoprofile, but no typical NLPHL nodules, no CD3+/CD57+ T-cell rosettes

    • Classic Hodgkin lymphoma: CD45-, CD20-, OCT2-, BOB1-, CD30+, CD15+, PAX5 weakly +, EBV+/-; may have T-cell rosettes, but T-cells CD57-

    • PTGC: lack of LH cells, be aware of reactive immunoblasts

    • ALCL: CD30+, CD2+, perforin/granzyme+, ALK+/-, CD43+/-, B-cell markers-.

    • Reactive lymphoid hyperplasia: especially when tumor cells are scant. Need to spend some time in higher power