ArticleAmerican journal of hematology2021
Validated international definition of the thrombocytopenia, anasarca, fever, reticulin fibrosis, renal insufficiency, and organomegaly clinical subtype (TAFRO) of idiopathic multicentric Castleman disease.
Article in American journal of hematology, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. It is linked to trial NCT02817997 (ACCELERATE), which is not on this map. Cited by 89 papers, 1 of them a synthesis that pooled it.
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The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
ACCELERATE (Advancing Castleman Care With an Electronic Longitudinal Registry, E-Repository, And Treatment/Effectiveness Research): An International Registry for Patients With Castleman Disease
Who cites it
89 citing papers in PubMed, 1 synthesis or guideline pooled it.
- The clinical picture of Castleman disease: a systematic review and meta-analysis.Blood advances · 2024Pooled it
- Successful treatment of idiopathic multicentric Castleman disease with TAFRO and kidney involvement: case report and literature review.Renal failure · 2026Review
- Idiopathic multicentric Castleman - TAFRO syndrome can mimic sepsis and myeloid neoplasia.Haematologica · 2026Article
- Rethinking Common Diagnoses: Idiopathic Multicentric Castleman Disease Presenting as TAFRO Syndrome: A Case Report.Clinical case reports · 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
- TAFRO syndrome complicating Sjögren's disease: response to subcutaneous tocilizumab.Clinical rheumatology · 2026Article
- Distinct interleukin-6 production in IPL and TAFRO subtypes of idiopathic multicentric Castleman disease.Haematologica · 2026Article
- Ruxolitinib in relapsed/refractory TAFRO syndrome: a report of two cases, including one positive for Epstein-Barr virus, and a literature review.International journal of hematology · 2026Review
- Comprehensive analysis of subtype-specific outcomes and management in Castleman disease: a 20-year cohort study.Blood advances · 2026Article
- Limited discriminatory performance of the iMCD-IPI in a Western cohort.The oncologist · 2026Article
- Elucidating the grading intricacies of idiopathic multicentric Castleman disease histopathology: a pathologist's perspective.American journal of clinical pathology · 2026Review
- Is "severe" idiopathic multicentric Castleman disease (iMCD)-idiopathic plasmacytic lymphadenopathy (IPL) really severe?HemaSphere · 2026Article
- The clinical significance of the Mediterranean fever gene MEFV variants in Castleman disease.Communications medicine · 2026Article
- Concurrent Idiopathic Multicentric Castleman Disease with TAFRO and Sjögren's Syndrome in a Young Patient: A Challenging Diagnostic Overlap.European journal of case reports in internal medicine · 2026Article
- Expert Perspective: Diagnosis and Treatment of Castleman Disease.Arthritis & rheumatology (Hoboken, N.J.) · 2026Review
- Case Report: Endothelial-targeted bridging therapy for a TTP-like phenotype in fulminant iMCD-TAFRO.Frontiers in immunology · 2026Article
- Clinical Utility of an Increased Pretreatment Immature Platelet Fraction in the Early Diagnosis of TAFRO Syndrome, Especially in Patients with Late-onset Thrombocytopenia: A Case Series and Literature Review.Internal medicine (Tokyo, Japan) · 2025Review
- Successful Treatment of Refractory TAFRO Syndrome with Cyclophosphamide: A Report of Two Cases.Internal medicine (Tokyo, Japan) · 2025Article
- International Consensus Histopathological Criteria for Subtyping Idiopathic Multicentric Castleman Disease Based on Machine Learning Analysis.American journal of hematology · 2025Article
29 more citing papers are in PubMed but not listed here.
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Authors and funding
16 authors.
Funding
Abstract
Thrombocytopenia, anasarca, fever, reticulin fibrosis, renal insufficiency, and organomegaly (TAFRO) syndrome is a heterogeneous entity manifesting with a constellation of symptoms described above that can occur in the context of idiopathic multicentric Castleman disease (iMCD) as well as infectious diseases, malignancies, and rheumatologic disorders. So, iMCD-TAFRO is an aggressive subtype of iMCD with TAFRO syndrome and often hyper-vascularized lymph nodes. Since we proposed diagnostic criteria of iMCD-TAFRO in 2016, we have accumulated new insights on the disorder and additional cases have been reported worldwide. In this systematic review and cohort analysis, we established and validated a definition for iMCD-TAFRO. First, we searched PubMed and Japan Medical Abstracts Society databases using the keyword "TAFRO" to extract cases. Patients with possible systemic autoimmune diseases and hematologic malignancies were excluded. Our search identified 54 cases from 50 articles. We classified cases into three categories: (1) iMCD-TAFRO (TAFRO syndrome with lymph node histopathology consistent with iMCD), (2) possible iMCD-TAFRO (TAFRO syndrome with no lymph node biopsy performed and no other co-morbidities), and (3) TAFRO without iMCD or other co-morbidities (TAFRO syndrome with lymph node histopathology not consistent with iMCD or other comorbidities). Based on the findings, we propose an international definition requiring four clinical criteria (thrombocytopenia, anasarca, fever/hyperinflammatory status, organomegaly), renal dysfunction or characteristic bone marrow findings, and lymph node features consistent with iMCD. The definition was validated with an external cohort (the ACCELERATE Natural History Registry). The present international definition will facilitate a more precise and comprehensive approach to the diagnosis of iMCD-TAFRO.
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