title: Castleman's disease of the spleen.
abstract: BACKGROUND
  Castleman's disease (CD), also known as giant or angiofolicular lymphoid hyperplasia or lymphoid hamartoma, is a group of atypical lymphoproliferative disorders that share common lymph node histological features and may be localized either to a single lymph node (unicentric) or occur systemically (multicentric).
  
  
  METHODS
  Herein, we present a rare case of a of 75-year-old female patient who was referred to our department and after a thorough work-up, underwent splenectomy with synchronous resection of an accessory spleen, splenic artery lymph nodes, and splenic hilar lymph nodes due to splenic involvement in a multicentric CD.
  
  
  RESULTS
  The pathology of the specimens led to the conclusion that it was a case of polycentric HHV-8-positive CD, affecting the spleen, the accessory spleen, and the lymph nodes.
  
  
  CONCLUSIONS
  Incidence of this rare condition is believed to be approximately 0.001-0.05%. CD has been linked to the human immunodeficiency virus (HIV), human herpes virus 8 (HHV-8), and is associated with malignancies. The pathogenesis mechanism is considered to be a dysregulation and hypersecretion of cytokines, either idiopathic or secondary to a viral infection, with the latter considered the most frequent. Solid organ involvement is very rare as is splenic involvement.
authors: a 2nd Department of Propedeutic Surgery , Athens Medical School, National and Kapodistrian University of Athens , Goudi, Athens , Greece.
authors: Dimitrios
authors: D
authors: Mantas
authors: a 2nd Department of Propedeutic Surgery , Athens Medical School, National and Kapodistrian University of Athens , Goudi, Athens , Greece.
authors: Christos
authors: C
authors: Damaskos
authors: b Department of Pathology , Athens Medical School, National and Kapodistrian University of Athens , Athens , Greece.
authors: Panagiota
authors: P
authors: Dailiani
authors: c 1st Department of Internal Medicine , Athens Medical School, National and Kapodistrian University of Athens , Athens , Greece.
authors: Michael
authors: M
authors: Samarkos
authors: b Department of Pathology , Athens Medical School, National and Kapodistrian University of Athens , Athens , Greece.
authors: Penelope
authors: P
authors: Korkolopoulou
mesh_terms: D000368:Aged; D005871:Castleman Disease / Q000175:diagnosis* / Q000601:surgery; D005260:Female; D006801:Humans; D013156:Splenectomy; D013158:Splenic Diseases / Q000175:diagnosis* / Q000601:surgery
keywords: Castleman’s disease; lymph nodes; spleen disorders