Table 1. Features of the different types of Castleman Disease.

Type of Castleman Disease	Type of Lymphadenopathy	Pathology	IL6 driven inflammatory syndromea	Virologic Status	Treatment
Unicentric	Localized	90% hyaline vascular	Typically not	Negative for HHV8 by QCPR or Negative LANA-1 stain	Complete Excision
Multicentric HHV8 positive	Generalized ±hepatosplenomegaly	plasmacytic or plasmablastic	Yes	Positive for HHV8 by QCPR; May be positive for HIV	Rituximab ±etoposide, Optional valganciclovir maintenance
Multicentric HHV8 negative	Generalized ±hepatosplenomegaly	Mostly plasmacytic, but can be hyaline vascular or mixed cellularity	Yes, but variable clinical presentation from mild to very severe and some cases do not respond to anti-IL-6 therapy	Negative for HHV8 by QCPR or Negative LANA-1 stain, Negative for HIV	Siltuximab, Tocilizimab, Rituximab, In severe cases Chemotherapy

^aSymptoms: fevers, night sweats, anorexia, weight loss, fatigue. Laboratory abnormalities: anemia, thrombocytopenia or thrombocytosis, elevated CRP, WESR, fibrinogen, hypergammaglobulinemia, abnormal renal function, increased IL6, VEGF, IL10.