https://doi.org/10.4081/itjm.2026.2528
Idiopathic multicentric Castleman disease: case report and literature review
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Published: 28 September 2026
Castleman disease is a rare disease characterized by lymphoproliferative disorders. We report the case of a 60-year-old man with diffuse lymphadenopathy, hepatosplenomegaly, and chylous ascites of unknown origin. A diagnosis of idiopathic multicentric Castleman disease (MCD) was performed, and the patient underwent treatment with cycles of rituximab, cyclophosphamide, and dexamethasone. Six months of treatment resulted in marked clinical and radiological improvement; maintenance therapy with rituximab alone was continued every 2 months. A review of the scientific literature shows that at least a third of all published cases of MCD are idiopathic. Corticosteroids, immunomodulatory or immunosuppressive agents, cytotoxic chemotherapy, and anti-interleukin-6 therapy have been used for patients with MCD. Timely diagnosis and appropriate treatment can significantly impact patient survival. Data on idiopathic MCD are limited, so MCD represents a clinical challenge because of the rarity of the disease and the lack of knowledge about it.
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