TAFRO syndrome caused by Castleman disease: a case report and literature review
10.3760/cma.j.cn441217-20240816-00814
- VernacularTitle:Castleman病引起的TAFRO综合征1例并文献复习
- Author:
Wenyuan LOU
1
;
Jingjing WANG
1
;
Duqun CHEN
1
;
Yuanmao TU
1
;
Dandan QIU
1
;
Zhen CHENG
1
;
Haitao ZHANG
1
Author Information
1. 南京大学医学院附属金陵医院(东部战区总医院)肾内科 国家肾脏疾病临床医学研究中心,南京 210016
- Publication Type:Journal Article
- Keywords:
Castleman disease;
Renal insufficiency;
Rituximab;
Lymph node biopsy;
TAFRO syndrome
- From:
Chinese Journal of Nephrology
2025;41(7):544-548
- CountryChina
- Language:Chinese
-
Abstract:
TAFRO syndrome is an idiopathic systemic inflammatory disease that overlaps with idiopathic multicentric Castleman disease (iMCD). The clinical features of TAFRO syndrome include thrombocytopenia (T), anasarca (A), fever (F), reticulin fibrosis/renal insufficiency (R) and organomegaly (O). The paper reports a special clinical subtype of iMCD—TAFRO syndrome in a patient, manifested as multiple-system involvement including serous effusion (ascites), fever, thrombocytopenia, anemia, multiple lymphadenopathies, pancreatitis and renal insufficiency. Bone marrow biopsy pathology showed active bone marrow hyperplasia. Renal biopsy revealed renal thrombotic microangiopathy, acute renal tubular interstitial injury combined with chronic lesions. Lymph node biopsy demonstrated lymphoproliferative lesions consistent with Castleman disease (hyaline vascular type). Following diagnosis, glucocorticoids, tacrolimus, rituximab and lenalidomide were administered, resulting in significant symptomatic improvement: ascites disappeared, and urinary findings, erythrocyte counts, renal function and hematological indexes normalized. The paper describes the patient's clinical manifestations, diagnosis and treatment process, and prognosis, and reviews relevant literature, to improve clinicians' understanding of this rare disease.