ArticleBlood2017
International, evidence-based consensus diagnostic criteria for HHV-8-negative/idiopathic multicentric Castleman disease.
Article in Blood, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 354 papers, 3 of them syntheses that pooled it.
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Who cites it
354 citing papers in PubMed, 3 syntheses or guidelines pooled it, 601 citations in OpenAlex.
- Australian clinical practice guideline: diagnosis and treatment of idiopathic multicentric Castleman disease.Internal medicine journal · 2026Guideline
- [Chinese guidelines on the diagnosis and treatment of adult essential thrombocythemia (2026)].Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi · 2026Guideline
- The clinical picture of Castleman disease: a systematic review and meta-analysis.Blood advances · 2024Pooled it
- A prospective study of zanubrutinib, a Bruton tyrosine kinase inhibitor, in relapsed/refractory idiopathic multicentric Castleman disease.International journal of hematology · 2024Trial
- Successful treatment of idiopathic multicentric Castleman disease with TAFRO and kidney involvement: case report and literature review.Renal failure · 2026Review
- Rethinking Common Diagnoses: Idiopathic Multicentric Castleman Disease Presenting as TAFRO Syndrome: A Case Report.Clinical case reports · 2026Article
- Eruptive gouty panniculitis associated with idiopathic multicentric Castleman disease (iMCD-NOS).JAAD case reports · 2026Article
- Idiopathic multicentric Castleman - TAFRO syndrome can mimic sepsis and myeloid neoplasia.Haematologica · 2026Article
- Sustained Remission of More Than Five Years After Thalidomide-Cyclophosphamide-Prednisone Discontinuation in Idiopathic Multicentric Castleman Disease: A Single-Center Retrospective StudyTurkish journal of haematology : official journal of Turkish Society of Haematology · 2026Article
- Idiopathic Multicentric Castleman Disease Associated With Seronegative Erosive Chronic Polyarthritis: A Diagnostic Challenge.Clinical case reports · 2026Article
- Grading of Castleman Disease Histopathology with an Attention-Based Multiple Instance Learning Model.Diagnostics (Basel, Switzerland) · 2026Article
- Development and Validation of the Idiopathic Multicentric Castleman Disease Symptom Burden Scale (ISBUS): Protocol for an International Multistage Mixed Methods Study.JMIR research protocols · 2026Article
- Real-World Usage and Outcomes of Different Siltuximab Dosing Frequency in Idiopathic Multicentric Castleman Disease.American journal of hematology · 2026Article
- Clinical feature analysis of pediatric Castleman disease with renal involvement.Pediatric nephrology (Berlin, Germany) · 2026Article
- TAFRO syndrome complicating Sjögren's disease: response to subcutaneous tocilizumab.Clinical rheumatology · 2026Article
- Systemic lupus erythematosus combined with Castleman disease: a case report.Wiener klinische Wochenschrift · 2026Article
- Morphological characteristics of the renal lesion in TAFRO syndrome and POEMS syndrome: a retrospective and multicenter study.Clinical and experimental nephrology · 2026Article
- Article
- Deciphering the full spectrum of Castleman diseases based on a cohort of 700 patients in a western country.British journal of haematology · 2026Article
- Interfollicular Plasmacytosis and Hyperplastic Germinal Centers in Idiopathic Multicentric Castleman Disease, Idiopathic Plasmacytic Lymphadenopathy Subtype.American journal of hematology · 2026Article
294 more citing papers are in PubMed but not listed here.
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Authors and funding
38 authors at 20 institutions in 9 countries.
Funding
Abstract
Human herpesvirus-8 (HHV-8)-negative, idiopathic multicentric Castleman disease (iMCD) is a rare and life-threatening disorder involving systemic inflammatory symptoms, polyclonal lymphoproliferation, cytopenias, and multiple organ system dysfunction caused by a cytokine storm often including interleukin-6. iMCD accounts for one third to one half of all cases of MCD and can occur in individuals of any age. Accurate diagnosis is challenging, because no standard diagnostic criteria or diagnostic biomarkers currently exist, and there is significant overlap with malignant, autoimmune, and infectious disorders. An international working group comprising 34 pediatric and adult pathology and clinical experts in iMCD and related disorders from 8 countries, including 2 physicians that are also iMCD patients, was convened to establish iMCD diagnostic criteria. The working group reviewed data from 244 cases, met twice, and refined criteria over 15 months (June 2015 to September 2016). The proposed consensus criteria require both Major Criteria (characteristic lymph node histopathology and multicentric lymphadenopathy), at least 2 of 11 Minor Criteria with at least 1 laboratory abnormality, and exclusion of infectious, malignant, and autoimmune disorders that can mimic iMCD. Characteristic histopathologic features may include a constellation of regressed or hyperplastic germinal centers, follicular dendritic cell prominence, hypervascularization, and polytypic plasmacytosis. Laboratory and clinical Minor Criteria include elevated C-reactive protein or erythrocyte sedimentation rate, anemia, thrombocytopenia or thrombocytosis, hypoalbuminemia, renal dysfunction or proteinuria, polyclonal hypergammaglobulinemia, constitutional symptoms, hepatosplenomegaly, effusions or edema, eruptive cherry hemangiomatosis or violaceous papules, and lymphocytic interstitial pneumonitis. iMCD consensus diagnostic criteria will facilitate consistent diagnosis, appropriate treatment, and collaborative research.
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