SHM Converge Scientific Abstract ePoster Gallery-Fever of Unknown Origin Reveals Multicentric Castleman Disease

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This case describes a 33-year-old previously healthy man with 3 weeks of high fevers, night sweats, rigors, fatigue, cognitive “fog,” and a 20-pound weight loss. Prior infectious testing and empiric antibiotics were unrevealing. On admission, CT imaging showed diffuse mediastinal, hilar, supraclavicular, axillary, and retroperitoneal lymphadenopathy, along with splenomegaly and pulmonary ground-glass opacities. A broad infectious, hematologic, and rheumatologic workup was negative except for a high ANA titer.<br /><br />An excisional inguinal lymph node biopsy showed paracortical hyperplasia with Castleman-like changes. After biopsy, serum IL-6 was elevated and HHV-8 testing was negative, supporting a diagnosis of multicentric Castleman disease (MCD). Surprisingly, the patient’s fevers and systemic symptoms gradually improved after lymph node removal alone, and the team chose to defer siltuximab because of continued clinical improvement.<br /><br />The presentation highlights key teaching points: Castleman disease is a rare lymphoproliferative disorder marked by lymph node hyperplasia and IL-6 overexpression. Unicentric Castleman disease involves one lymph node region and is often cured by surgical excision, whereas multicentric disease affects multiple nodal stations and causes systemic inflammation. Treatment of MCD depends on HHV-8 status and usually involves IL-6–targeted therapy or monoclonal antibodies such as rituximab.<br /><br />This case is notable because the patient improved without standard pharmacologic therapy after excision of two lymph nodes, raising questions about the mechanisms of remission. For hospitalists, Castleman disease should be included in the differential diagnosis of lymphoma-like illness with unexplained fever and diffuse lymphadenopathy.

Asset Subtitle

James Casola

Meta Tag

Author List Anisha Advani, James Casola

Category Clinical Vignettes

Concept Idiopathic Multicentric Castleman Disease

Concept Lymphadenopathy

Concept Interleukin-6

Concept Unicentric Castleman Disease

Concept Human Herpesvirus 8

Distinguished Non-Finalist

Presenter Organization Yale New Haven Health

Presenting Author James Casola

Track Adult

Keywords

Castleman disease

multicentric Castleman disease

lymphadenopathy

interleukin-6

HHV-8 negative

fever of unknown origin

splenomegaly

lymph node biopsy

siltuximab

rituximab

Idiopathic Multicentric Castleman Disease

Lymphadenopathy

Interleukin-6

Unicentric Castleman Disease

Human Herpesvirus 8