Multicentric Castleman disease (MCD) is a rare, systemic lymphoproliferative disorder characterized by widespread lymph node enlargement and excessive immune system activation. Unlike unicentric Castleman Disease (UCD), which affects a single lymph node region, MCD involves multiple lymph node stations and can lead to significant systemic symptoms.
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Of note, idiopathic MCD (iMCD) has been associated with features of excessive IL-6 production, leading to systemic inflammation, and can be associated with autoimmune-like manifestations and paraneoplastic syndromes. We came across a pair of identical twins manifesting features initially suggestive of a polyclonal gammopathy of unknown cause - subsequently worked up to be consistent with iMCD. Through a patient-partnership approach, further research uncovered putative mutations in their DNA make-up that could explain this unusual condition. In addition, we identified the cell-of-origin of IL-6 signals to be emanating from specific stromal cells of the lymph node.
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Link to full publication below:
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Commentary by Blood journal:
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van Rhee F, Fajgenbaum D. Insights into the etiology of Castleman disease. Blood. 2024 May 2;143(18):1789-1790. doi: 10.1182/blood.2023023716. PMID: 38696194.
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