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Case 86 - Discussion

Uploaded: 2009-08-20, Updated: 2009-08-20

 

Castleman's Disease

  • Two major histological types: hyaline-vascular and plasma cell. IL6 important in the pathogenesis.

  • Hyaline vascular type

    • Younger age, M=F, mostly in the mediastinum and asymptomatic.

    • mantle cell hyperplasia, concentric layering, onion skinning.

    • Vessels radially penetrating into germinal center, lollipop.

    • Lymphocyte depleted germinal centers with most of the cells being follicular dendritic cells.

    • Collection of CD123+ plasmacytoid cells in the interfollicular areas.

  • Plasma cell type

    • Less common, ~15%, older age, more symptomatic.

    • Sheets of interfollicular plasma cells, may show follicular hyperplasia.

  • Multicentric Castleman disease

    • Aggressive, poor prognosis.

    • Mostly plasma cell type.

    • Associated with POEMS syndrome.

    • HHV8 associated (also PEL, Kapposi and PBL).