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Case 200 - Discussion

Uploaded: 2011-09-22, Updated: 2011-09-22

 

Castleman's Disease, Plasma Cell Variant

  • Solitary type: a solitary mass composed of multiple discrete lymph nodes
  • Multicentric or systemic type:
    • HIV associated
    • Generalized adenopathy and involvement of spleen
    • Systemic symptoms (fever, anemia) and laboratory abnormalities (elevated erythrocyte sedimentation rate, hypergammaglobulinemia or hypoalbuminemia)
    • Seen in older individuals and is frequently aggressive
    • Associated with the POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, M protein and skin changes), amyloid deposits
    • HHV-8 infection
  • Abundant expression of interleukin-6, responsible for the plasma cell infiltration.
  • Microscopically, the lymph nodes demonstrate extensive architectural obliteration by sheets of plasma cells between hyperplastic follicular centers; interfollicular vascular proliferation; the sinuses are usually preserved; lack of hyaline-vascular changes
  • A reactive disorder may have monoclonal but polytypic plasma cells