ArticleBritish journal of haematology2024
Immune-mediated thrombocytopenia and IL-6-mediated thrombocytosis observed in idiopathic multicentric Castleman disease.
Article in British journal of haematology, 2024. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 10 papers.
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Who cites it
10 citing papers in PubMed, 8 citations in OpenAlex.
- Serum interleukin-6 (Il-6) monitoring in idiopathic multicentric Castleman disease: important value in patients receiving non-anti-Il-6 therapy.Biomarker research · 2026Article
- Distinct interleukin-6 production in IPL and TAFRO subtypes of idiopathic multicentric Castleman disease.Haematologica · 2026Article
- Multicentric Castleman disease (MCD) presenting with retiform purpura: A rare dermatologic manifestation of a systemic disease.JAAD case reports · 2026Article
- Serum Soluble Interleukin-2 Receptor as a Potential Marker for Assessing Disease Activity in the TAFRO Subtype of Idiopathic Multicentric Castleman Disease.Internal medicine (Tokyo, Japan) · 2025Article
- Response to initial treatment with glucocorticoids in TAFRO syndrome and implications for secondary treatment.International journal of hematology · 2025Article
- Successful treatment of a chronic myeloid leukemia patient with extreme thrombocytosis by a combination of imatinib and interferon‑α: A case report.Experimental and therapeutic medicine · 2025Article
- Common connective tissue disorder and anti-cytokine autoantibodies are enriched in idiopathic multicentric castleman disease patients.Frontiers in immunology · 2025Article
- Dynamic changes in platelet counts and psychological state in ITP patients after COVID-19 infection.Frontiers in medicine · 2025Article
- Monocarboxyoctyl phthalate is associated with platelet count: evidence from a large cross-sectional study.Frontiers in public health · 2025Article
- TAFRO Syndrome: Guidance for Managing Patients Presenting Thrombocytopenia, Anasarca, Fever, Reticulin Fibrosis, Renal Insufficiency, and Organomegaly.Biomedicines · 2024Review
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Authors and funding
9 authors at 1 institution in 1 country.
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Abstract
Idiopathic multicentric Castleman disease (iMCD) is a rare haematological disorder characterized by generalized lymphadenopathy with atypical histopathological features and systemic inflammation caused by a cytokine storm involving interleukin-6 (IL-6). Three clinical subtypes are recognized: thrombocytopenia, anasarca, fever, renal dysfunction, organomegaly (iMCD-TAFRO); idiopathic plasmacytic lymphadenopathy (iMCD-IPL), involving thrombocytosis and hypergammaglobulinaemia; and iMCD-not otherwise specified (iMCD-NOS), which includes patients who do not meet criteria for the other subtypes. Disease pathogenesis is poorly understood, with potential involvement of infectious, clonal and/or autoimmune mechanisms. To better characterize iMCD clinicopathology and gain mechanistic insights into iMCD, we analysed complete blood counts, other clinical laboratory values and blood smear morphology among 63 iMCD patients grouped by clinical subtype. Patients with iMCD-TAFRO had large platelets, clinical severity associated with lower platelet counts and transfusion-resistant thrombocytopenia, similar to what is observed with immune-mediated destruction of platelets in immune thrombocytopenic purpura. Conversely, elevated platelet counts in iMCD-IPL were associated with elevated IL-6 and declined following anti-IL-6 therapy. Our data suggest that autoimmune mechanisms contribute to the thrombocytopenia in at least a portion of iMCD-TAFRO patients whereas IL-6 drives thrombocytosis in iMCD-IPL, and these mechanisms likely contribute to disease pathogenesis.
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