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  • Surgical Pathology Criteria
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    KSHV-associated Germinotropic Lymphoproliferative Disorder

    Definition

    • KSHV (HHV8) associated lymphoproliferative disorder with plasmablasts involving germinal centers

    Diagnostic Criteria

    • Plasmablastic cytology
      • 1 or 2 prominent nucleoli
      • Moderate amount of amphophilic cytoplasm
    • Plasmablasts preferentially involve germinal centers
      • Partial or complete involvement
      • Clusters or aggregates
    • Plasmablasts HHV8 (KSHV) and EBV positive
    • Remainder of node not involved
      • Germinal centers show no features of Castleman disease
      • Prominent interfollicular plasmacytosis
        • No interfollicular plasmablasts
    • One published report of three patients by Du et al.

    Yasodha Natkunam MD PhD
    Robert V Rouse MD
    Department of Pathology
    Stanford University School of Medicine
    Stanford CA 94305-5342

    Original posting:: May 1, 2006

    Supplemental studies

    Immunohistology

    • HHV8 positive
    • B lineage negative
    • CD38 2/3
    • CD30 1/3
    • Light chain monotypic

    Genetic analysis

    • EBV EBER positive
    • Oligoclonal or polyclonal (one case each)
      • Third case not analyzable

    Differential Diagnosis

     

    Plasmablastic Lymphoma KSHV-associated Germinotropic Lymphoproliferative Disorder
    Typically oral cavity Localized lymphadenopathy
    Immunosupressed patient Immunocompetent patient
    Sheets of plasmablasts Plasmablasts involve germinal centers
    Some cases associated with multicentric Castleman disease (MCD) No Castleman features in follicles
    EBV 50% positive EBV positve
    Plasmablasts are the lesional cells in both disorders and both may be HHV8 positive


    Both may partially replace the node and be negative or minimally reactive for CD20 and CD45RB
    Diffuse Large B Cell Lymphoma KSHV-associated Germinotropic Lymphoproliferative Disorder
    Typically effaced architecture Overall nodal architecture preserved
    Typically sheets of large atypical cells Follicular pattern
    HHV8 and EBV negative except in immunosuppressed patients HHV8 and EBV positive


    Anaplastic Large Cell Lymphoma KSHV-associated Germinotropic Lymphoproliferative Disorder
    Sinusoidal pattern Follicular pattern
    ALK positive 60-85% ALK negative
    HHV8 and EBV negative HHV8 and EBV positive
    T lineage (T cell receptor) B lineage (light chain expression)
    Both share cytologic features, may partially replace the node with clusters of large atypical cells and may be negative or minimally reactive for CD45RB

    Clinical

    • 3 cases reported, age 41-63
    • Not immunosupressed
    • Localized lymphadenopathy
    • Good response to chemotherapy in two treated cases

    Grading / Staging / Report

    • Grading and staging not applicable

    The pathology report should contain the following information:

    • Refer to publication on this rare subject
    • Results of supplementary studies if performed

     

    Lists

    HHV8 (KSHV) positive processes

    Bibliography

    • Warnke RA, Weiss LM, Chan JKC, Cleary ML, Dorfman R . Tumors of the Lymph Nodes and Spleen, Atlas of Tumor Pathology, AFIP Third Series, Fascicle 14, 1995
    • Jaffe ES, Harris NL Stein H, Vardiman JW eds. Pathology and Genetics of Tumours of Haematopoietic and Lymphoid Tissues, World Health Organization Classification of Tumours 2001
    • Du MQ, Diss TC, Liu H, Ye H, Hamoudi RA, Cabecadas J, Dong HY, Harris NL, Chan JK, Rees JW, Dogan A, Isaacson PG. KSHV- and EBV-associated germinotropic lymphoproliferative disorder. Blood. 2002 Nov 1;100(9):3415-8.
    Printed from Surgical Pathology Criteria: http://surgpathcriteria.stanford.edu/
    © 2005  Stanford University School of Medicine