BACKGROUND: Castleman disease is an uncommon lymphoproliferative disorder 
characterized as either unicentric or multicentric. Unicentric Castleman disease 
(UCD) is localized and carries an excellent prognosis, whereas multicentric 
Castleman disease (MCD) is a systemic disease occurring most commonly in the 
setting of HIV infection and is associated with human herpesvirus 8. MCD has 
been associated with considerable morbidity and mortality, and the therapeutic 
landscape for its management continues to evolve.
METHODS: The available medical literature on UCD and MCD was reviewed. The 
clinical presentation and pathological diagnosis of Castleman disease was 
reviewed, along with associated disorders such as certain malignancies and 
autoimmune complications.
RESULTS: Surgical resection remains the standard therapy for UCD, while systemic 
therapies are required for the management of MCD. Rituximab monotherapy is the 
mainstay of therapy; however, novel therapies targeting interleukin 6 may 
represent a treatment option in the near future. Antiviral strategies as well as 
single-agent and combination chemotherapy with glucocorticoids are established 
systemic therapies. The management of Castleman disease also requires careful 
attention to potential concomitant infections, malignancies, and associated 
syndromes.
CONCLUSIONS: UCD and MCD constitute uncommon but well-defined clinicopathologic 
entities. Although UCD is typically well controlled with local therapy, MCD 
continues to pose formidable challenges in management. We address historical 
chemotherapy-based approaches to this disease as well as recently developed 
targeted therapies, including rituximab and siltuximab, that have improved the 
outcome for newly diagnosed patients. Ongoing research into the management of 
MCD is needed.