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).