Unicentric Castleman Disease — SCE Infectious Diseases MCQ

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HardHIV MedicineUnicentric Castleman DiseaseSCE Infectious Diseases

A 30-year-old man with HIV (CD4 280, VL <50 on ART) develops a single painless inguinal lymph node. FNA is non-diagnostic. Excisional biopsy shows Castleman disease (unicentric, hyaline vascular variant). HHV-8 testing is negative. What is the management?

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Correct answer: B — Surgical excision is curative for unicentric Castleman disease — this is not an infectious disease but the HHV-8 association should be assessed (negative here)

Unicentric Castleman disease (UCD) is a lymphoproliferative disorder that presents as a solitary enlarged lymph node. The hyaline vascular variant is the most common. UCD is usually HHV-8-negative (unlike multicentric Castleman disease — MCD — which is HHV-8-positive and HIV-associated). Surgical excision is curative for UCD with >90% long-term disease-free survival. MCD in HIV is a different entity: HHV-8-driven, multisystemic, and treated with Rituximab ± Etoposide. The distinction between UCD and MCD is critical.

Reference: BHIVA 2022 – HIV-associated lymphoproliferation; BSH 2023 – Castleman disease