Kidney involvement in idiopathic multicentric Castleman disease-TAFRO syndrome: clinical spectrum, pathology and outcomes.
- 1Department of Nephrology, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing Key Laboratory of Molecular Pathology Diagnosis and Translational Research on Rare and Complex Urinary System Diseases, Beijing, China.
- 2Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College, Beijing,China.
Abstract
Background
Idiopathic multicentric Castleman disease-thrombocytopenia, anasarca, myelofibrosis, renal dysfunction and organomegaly syndrome (iMCD-TAFRO) is a clinically aggressive subtype. Renal involvement and its impact on prognosis are poorly characterized. We aimed to describe renal clinicopathological features and outcomes in a large iMCD-TAFRO cohort.Methods
This 10-year retrospective cohort study enrolled iMCD-TAFRO patients at Peking Union Medical College Hospital. Renal involvement was defined by iMCD consensus criteria. Survival was analyzed using Kaplan-Meier methods.Results
A total of 48 patients (70.8% male; mean age 45 ± 15 years) were included. Renal involvement was observed in 95.8% (46/48) of the patients, presenting predominantly with reduced urine output, mild hematuria and proteinuria, as well as elevated serum creatinine. Acute kidney injury (AKI) occurred in 80.4% (37/46) of patients showing renal involvement and 37.8% of them required renal replacement therapy. Renal biopsies (n = 7) revealed glomerular microangiopathy in six cases. AKI was associated with elevated C-reactive protein (CRP) and D-dimer levels, and CRP levels correlated with peak serum creatinine levels. Therapy targeting the iMCD was mainly bortezomib, cyclophosphamide and dexamethasone regimen (31/48, 64.6%) with/without anti-interleukin-6 therapy. Nine patients died within hospital or during follow-up. Among AKI patients, 28/37 (75.7%) showed full recovery of renal function. Despite worse renal function at baseline, no significant survival difference was observed between dialyzed and non-dialyzed patients.Conclusions
Renal involvement in iMCD-TAFRO is common and frequently manifests as AKI associated with systemic inflammation. Early and aggressive therapy combined with supportive renal care, including timely dialysis, improved renal recovery and overall prognosis.
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BibTeX
@article{zhang2026kidney,
title = {{Kidney involvement in idiopathic multicentric Castleman disease-TAFRO syndrome: clinical spectrum, pathology and outcomes.}},
author = {Shuo Zhang and Hanshu Liu and Siyuan Li and Yubing Wen and Wenling Ye and Wei Ye and Hua Zheng and Lubin Xu and Fei Teng and Ying Wang and Ke Zheng and Peng Xia and Lu Zhang and Jian Li and Xuemei Li and Limeng Chen},
journal = {Clinical kidney journal},
year = {2026},
month = sep,
doi = {10.1093/ckj/sfag326},
url = {https://europepmc.org/article/MED/42898812},
note = {CC BY-NC},
}
RIS
TY - JOUR
TI - Kidney involvement in idiopathic multicentric Castleman disease-TAFRO syndrome: clinical spectrum, pathology and outcomes.
AU - Shuo Zhang
AU - Hanshu Liu
AU - Siyuan Li
AU - Yubing Wen
AU - Wenling Ye
AU - Wei Ye
AU - Hua Zheng
AU - Lubin Xu
AU - Fei Teng
AU - Ying Wang
AU - Ke Zheng
AU - Peng Xia
AU - Lu Zhang
AU - Jian Li
AU - Xuemei Li
AU - Limeng Chen
PY - 2026
DA - 2026/09/14
JO - Clinical kidney journal
DO - 10.1093/ckj/sfag326
UR - https://europepmc.org/article/MED/42898812
AB - Background Idiopathic multicentric Castleman disease-thrombocytopenia, anasarca, myelofibrosis, renal dysfunction and organomegaly syndrome (iMCD-TAFRO) is a clinically aggressive subtype. Renal involvement and its impact on prognosis are poorly characterized. We aimed to describe renal clinicopathological features and outcomes in a large iMCD-TAFRO cohort.Methods This 10-year retrospective cohort study enrolled iMCD-TAFRO patients at Peking Union Medical College Hospital. Renal involvement was defined by iMCD consensus criteria. Survival was analyzed using Kaplan-Meier methods.Results A total of 48 patients (70.8% male; mean age 45 ± 15 years) were included. Renal involvement was observed in 95.8% (46/48) of the patients, presenting predominantly with reduced urine output, mild hematuria and proteinuria, as well as elevated serum creatinine. Acute kidney injury (AKI) occurred in 80.4% (37/46) of patients showing renal involvement and 37.8% of them required renal replacement therapy. Renal biopsies (n = 7) revealed glomerular microangiopathy in six cases. AKI was associated with elevated C-reactive protein (CRP) and D-dimer levels, and CRP levels correlated with peak serum creatinine levels. Therapy targeting the iMCD was mainly bortezomib, cyclophosphamide and dexamethasone regimen (31/48, 64.6%) with/without anti-interleukin-6 therapy. Nine patients died within hospital or during follow-up. Among AKI patients, 28/37 (75.7%) showed full recovery of renal function. Despite worse renal function at baseline, no significant survival difference was observed between dialyzed and non-dialyzed patients.Conclusions Renal involvement in iMCD-TAFRO is common and frequently manifests as AKI associated with systemic inflammation. Early and aggressive therapy combined with supportive renal care, including timely dialysis, improved renal recovery and overall prognosis.
SN - 2048-8505
SN - 2048-8513
ER -