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Castleman's disease of the spleen

Dimitrios Mantas1, Christos Damaskos1, Panagiota Dailiani2

  • 1a 2nd Department of Propedeutic Surgery , Athens Medical School, National and Kapodistrian University of Athens , Goudi, Athens , Greece.

Acta Chirurgica Belgica
|October 25, 2016
PubMed

Insights

This study reports a rare case of multicentric Castleman

Area of Science:

  • Hematology
  • Oncology
  • Pathology

Background:

  • Castleman's disease (CD) is a rare lymphoproliferative disorder with unicentric or multicentric presentations.
  • It is associated with human herpes virus 8 (HHV-8), human immunodeficiency virus (HIV), and malignancies.
  • Cytokine dysregulation is a key pathogenic mechanism, often triggered by viral infections.

Observation:

  • A 75-year-old female presented with multicentric Castleman's disease.
  • The patient underwent splenectomy for splenic involvement, including accessory spleen and hilar lymph nodes.
  • Pathology confirmed polycentric HHV-8-positive Castleman's disease affecting the spleen and lymph nodes.

Findings:

  • Splenic and accessory spleen involvement in Castleman's disease is exceptionally rare.
  • This case highlights HHV-8-positive Castleman's disease with extensive splenic and nodal involvement.
  • The polycentric nature of the disease was confirmed through histopathological examination.

Implications:

  • This case underscores the importance of considering Castleman's disease in the differential diagnosis of splenic masses.
  • Early diagnosis and management are crucial due to potential associations with malignancies.
  • Further research into the pathogenesis and treatment of rare CD presentations is warranted.
Abstract

Keywords:
Castleman’s diseaselymph nodesspleen disorders

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