Abstract
Idiopathic multicentric Castleman disease is a rare lymphoproliferative disorder characterised by lymphadenopathy and systemic symptoms, in some cases leading to organ failure. The condition must be distinguished from unicentric Castleman disease, which is generally curable with local therapy, and HHV-8-associated Castleman disease, for which an effective treatment approach has been defined. 1 Fajgenbaum DC Uldrick TS Bagg A et al. International, evidence-based consensus diagnostic criteria for HHV-8-negative/idiopathic multicentric Castleman disease. Blood. 2017; 129: 1646-1657 Crossref PubMed Scopus (171) Google Scholar , 2 Bower M How I treat HIV-associated multicentric Castleman disease. Blood. 2010; 116: 4415-4421 Crossref PubMed Scopus (87) Google Scholar , 3 Fajgenbaum DC Novel insights and therapeutic approaches in idiopathic multicentric Castleman disease. Blood. 2018; 132: 2323-2330 Crossref PubMed Scopus (26) Google Scholar A hallmark of idiopathic multicentric Castleman disease is hypersecretion of cytokines, particularly IL6, the triggering factors for which might be multiple, leading to a common pathway of cytokine derangement. 3 Fajgenbaum DC Novel insights and therapeutic approaches in idiopathic multicentric Castleman disease. Blood. 2018; 132: 2323-2330 Crossref PubMed Scopus (26) Google Scholar Although lymphocytes are non-clonal, the clinical spectrum ranges from non-severe to life-threatening. Long-term safety of siltuximab in patients with idiopathic multicentric Castleman disease: a prespecified, open-label, extension analysis of two trialsThese results show that siltuximab is well tolerated long term and provides important evidence for the feasibility of the life-long use required by patients with idiopathic multicentric Castleman disease. Full-Text PDF
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