Pearls
Castleman Disease

• Castleman Disease is a rare B-cell lymphoproliferation disorder
  
• Castleman Disease occurs in two forms: the more common Localized Castleman Disease often presents as an asymptomatic mediastinal mass, wheras Multicentric Castleman Disease usually presents as a multisystem illness with sidespread lymphadenopathy.
• The diagnosis of Castleman Disease rests upon both the characteristic lymph node histology and the exclusion of other disorders.
• The pathogenesis of Castleman Disease appears to involve dysregulation of IL-6.
• Localized Castleman Disease usually is cured by surgical removal of the mediastinal mass.  Multicentric Castleman Disease has a poor prognosis, but may respond to prednisone, chemotherapy or surgical removal of affected nodes and spleen.