Castleman disease is a heterogeneous lymphoproliferative disorder that includes unicentric (UCD) and multicentric (MCD) forms; MCD can be driven by HHV-8 or be HHV-8-negative (idiopathic, iMCD). Diagnosis can be challenging and management differs by subtype. Case presentation: We present a de-identified adult patient with progressive constitutional symptoms, anemia, polyclonal hypergammaglobulinemia, and generalized lymphadenopathy. Lymph node biopsy showed characteristic features consistent with plasma-cell-rich iMCD. Infectious, autoimmune, and malignant causes were excluded; HHV-8 testing was negative. The patient received corticosteroids and anti-IL-6 therapy (siltuximab) with clinical and laboratory improvement. Conclusions: Early recognition and subtype classification of Castleman disease is essential because UCD is typically cured by surgical excision, while MCD often requires systemic therapy directed at IL-6 or B-cells depending on HHV-8 status. Recent consensus diagnostic criteria and treatment guidelines inform workup and management.
Voicu et al. (Tue,) studied this question.