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Immunodeficiency-associated lymphoproliferative disorders: time for reappraisal?

Yasodha Natkunam, Dita Gratzinger, Amy Chadburn, John R. Goodlad, John K. C. Chan, Jonathan Said, Elaine S. Jaffe and Daphne de Jong

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Tables

  • Table 1.

    Proposed unifying nomenclature and examples of immunodeficiency-associated LPDs

    3-Part unifying nomenclature
    Name of lesionViral statusSpecific immunodeficiency setting
    B-cell hyperplasia (eg, plasmacytic hyperplasia)eg, EBV+/−, HHV8+/−eg, Posttransplant (solid organ), iatrogenic (methotrexate), immune senescence
    Polymorphic B-cell lymphoproliferations (eg, mucocutaneous ulcer)
    Lymphoma (WHO terminology) (eg, diffuse large B-cell lymphoma, Anaplastic large cell lymphoma, ALK)
    Examples of diagnostic labels
    Proposed unifying nomenclatureWHO 2016 nomenclature
    Plasmacytic hyperplasia, EBV+, posttransplant (solid organ)Plasmacytic hyperplasia, nondestructive posttransplant LPD
    Polymorphic B-LPD, EBV+, iatrogenic (methotrexate)Polymorphic LPD resembling polymorphic posttransplant LPD
    Mucocutaneous ulcer, EBV+, primary immunodeficiency (CHARGE syndrome)EBV+ mucocutaneous ulcer
    Diffuse large B-cell lymphoma, EBV, HIV infectionDiffuse large B-cell lymphoma
    Diffuse large B-cell lymphoma, T-cell/histiocyte-rich, EBV+, immune senescenceEBV+ diffuse large B-cell lymphoma
    Primary effusion lymphoma, HHV8+, EBV+, HIV infectionPrimary effusion lymphoma
  • Table 2.

    Immunodeficiency-associated EBV+ B-cell LPDs

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  • Table 3.

    Immunodeficiency-associated HHV8+ LPDs

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