ArticleKidney international reports2020
The Clinical and Histopathological Feature of Renal Manifestation of TAFRO Syndrome.
Article in Kidney international reports, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 29 papers, 1 of them a synthesis that pooled it.
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Who cites it
29 citing papers in PubMed, 1 synthesis or guideline pooled it, 33 citations in OpenAlex.
- Kidney biopsy guidebook 2020 in Japan.Clinical and experimental nephrology · 2021Guideline
- Clinicopathological features and outcomes of glomerular microangiopathy: a retrospective cohort study.Renal failure · 2026Article
- Successful treatment of idiopathic multicentric Castleman disease with TAFRO and kidney involvement: case report and literature review.Renal failure · 2026Review
- Kidney involvement in idiopathic multicentric Castleman disease-TAFRO syndrome: clinical spectrum, pathology and outcomes.Clinical kidney journal · 2026Article
- Morphological characteristics of the renal lesion in TAFRO syndrome and POEMS syndrome: a retrospective and multicenter study.Clinical and experimental nephrology · 2026Article
- Kidney biopsy analysis of 14 cases of renal disease after unrelated cord blood transplantation.BMC nephrology · 2026Article
- Ultrasound-guided lateral kidney biopsy in patients unsuitable for the prone position.Clinical kidney journal · 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
- First-line treatment of TAFRO syndrome with rituximab: a case report and literature review.Annals of hematology · 2025Review
- Article
- TAFRO Syndrome Without Pathology Supporting Castleman Disease: To Be Treated as Idiopathic Multicentric Castleman Disease-TAFRO or a Distinct Disease Entity?Turkish journal of haematology : official journal of Turkish Society of Haematology · 2025Article
- Renal involvement in TAFRO syndrome: a review.Clinical and experimental nephrology · 2025Review
- Long-term clinicopathological characteristics of TAFRO syndrome and its relapse: a case series study.Clinical kidney journal · 2024Article
- A case of TAFRO syndrome after vaccination, successfully treated with cyclosporine.BMC nephrology · 2024Article
- TAFRO Syndrome: Guidance for Managing Patients Presenting Thrombocytopenia, Anasarca, Fever, Reticulin Fibrosis, Renal Insufficiency, and Organomegaly.Biomedicines · 2024Review
- Unraveling TAFRO Syndrome: An In-Depth Look at the Pathophysiology, Management, and Future Perspectives.Biomedicines · 2024Review
- The experience of diagnosis and treatment for TAFRO syndrome.Annals of hematology · 2023Article
- Severe TAFRO Syndrome Mimicking Hepatorenal Syndrome Successfully Treated with a Multidisciplinary Approach: A Case Report and Literature Review.Internal medicine (Tokyo, Japan) · 2023Review
- Adolescent-onset TAFRO Syndrome with Malignant Nephrosclerosis-like Lesions.Internal medicine (Tokyo, Japan) · 2023Article
- Siltuximab Monotherapy in Tafro Syndrome: A Case Report and Review of the Literature.Journal of nephrology · 2023Review
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Authors and funding
17 authors at 3 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
introductionThrombocytopenia, anasarca, myelofibrosis, renal dysfunction, and organomegaly (TAFRO) syndrome is a severe subtype of idiopathic multicentric Castleman's disease, characterized by thrombocytopenia, anasarca, fever, reticulin myelofibrosis, and organomegaly. Renal complication of this disease can be life-threatening and sometimes requires hemodialysis, but it has not been elucidated in detail.
methodsCase-series was designed to evaluate the renal histology of patients with TAFRO syndrome treated at our hospital.
resultsSeven patients were eligible to the criteria. All of them had severe diuretic-resistant anasarca and 6 of 7 had mild proteinuria (<1 g daily). On light microscopy, all patients showed glomerular endotheliopathy characterized by endothelial cell swelling and a double contour of the glomerular basement membrane with mesangiolysis or mesangial loosening. Immunofluorescent staining and electron microscopy did not detect immune deposits in any patient. Electron microscopy revealed endothelial cell swelling with diffuse expansion of the subendothelial space, loss of mesangial architecture, and loss of endothelial cell fenestrations. Treatment with glucocorticoids and molecular-targeting agents, including tocilizumab and rituximab, improved renal dysfunction and anasarca. In 4 of 7 patients with persistent thrombocytopenia, hemorrhagic events occurred despite platelet transfusion or thrombopoietin receptor antagonist therapy.
conclusionSevere diuretic-resistant anasarca with mild proteinuria and severe glomerular endotheliopathy were common characteristics of renal dysfunction due to TAFRO syndrome. In addition, endothelial changes mediated via interleukin (IL)-6 and vascular endothelial growth factor (VEGF) that lead to vascular hyperpermeability and water leakage might contribute to anasarca, because molecular-targeting therapy directed against IL-6 or VEGF improved renal dysfunction and severe endothelial damage.
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