Clinical kidney journal · 2026
Kidney involvement in idiopathic multicentric Castleman disease-TAFRO syndrome: clinical spectrum, pathology and outcomes.
СтатьяНазвание и аннотация: Перевод пока не подготовлен
Обработка статьи · этапы, ошибки и управление
Загрузка состояния…
Ошибки и ограничения
История · было → стало
Состояние обновляется каждые 5 секунд, пока блок открыт. Процент показывается только там, где известен объём работы. Пауза останавливает продолжение этапа; текущий запрос к источнику может завершиться.
Zhang Shuo, Liu Hanshu, Li Siyuan, Wen Yubing, Ye Wenling, Ye Wei, Zheng Hua, Xu Lubin, Teng Fei, Wang Ying, Zheng Ke, Xia Peng, Zhang Lu, Li Jian, Li Xuemei, Chen Limeng
Аннотация
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.
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.