systemic polyclonal immunoblastic proliferation


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  • Clinical

    • site: blood, marrow, node, organs
    • lymphocytosis with reactive lymphs, immunoblasts, plasma cells
    • can have neutrophilia with left shift
    • anemia w + direct coombs test, thrombocytopenia almost always present
    • often w polyclonal hypergammaglobulinemia
    • tx: steroid, chemo
    • 50% mortality during acute phase

  • Morphology

    • marrow: lymphs, immunoblasts, plasma cells, focal lymphocytic aggregates

  • Immunophenotype

    • polytypic plasma cells

  • Molecular

    • negative for IGH or TCR rearrangement

  • Ddx

    • angioimmunoblastic T cell lymphoma
    • EBV associated lymphoproliferative disorders
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