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[Unicentric Castleman disease: a blind lymph node.]

Sara Rizzi1, Ilaria Polenzani1, Angela Troisi2

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A rare case of unicentric Castleman

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Area of Science:

  • Pediatric Hematology
  • Oncology
  • Immunology

Background:

  • Castleman's disease is a rare lymphoproliferative disorder.
  • Unicentric Castleman's disease (UCD) typically presents as a localized lymphadenopathy.

Observation:

  • A 13-year-old female presented with persistent fever, mild anemia, and elevated inflammatory markers.
  • Imaging revealed para-aortic lymphadenopathy, diagnosed as unicentric Castleman's disease.
  • Surgical resection was incomplete, and inflammatory markers persisted post-operatively.

Findings:

  • Histopathological examination confirmed unicentric Castleman's disease.
  • Despite surgery, persistent high C-reactive protein (CRP), erythrocyte sedimentation rate (ERS), and serum amyloid A levels were noted.
  • Treatment with tocilizumab, an anti-interleukin-6 (IL-6) agent, led to complete remission of inflammatory indices.

Implications:

  • This case highlights the diagnostic challenges of Castleman's disease in adolescents.
  • Anti-IL-6 therapy is a viable and effective treatment option for symptomatic, unresectable unicentric Castleman's disease.
  • Early consideration of targeted therapies like tocilizumab can improve patient outcomes.