Abstract: SA-PO0756
The Clue Is in the Kidney: A Kidney Biopsy Diagnosis of TAFRO Syndrome
Session Information
- Glomerular Diseases: Lupus Nephritis, Monoclonal Gammopathy-Related Disease, and Transplantation
October 24, 2026 | Location: Exhibit Hall A, Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Glomerular Diseases
- 1402 Glomerular Diseases: Clinical, Outcomes, and Therapeutics
Authors
- Hadari, Itai, Montefiore Einstein Medical Center, New York, New York, United States
- Corona, Antonio Gabriel De Leon, Montefiore Einstein Medical Center, New York, New York, United States
- Pullman, James M., Montefiore Einstein Medical Center, New York, New York, United States
- Dominguez, Emma C., Ursuline School, New Rochelle, New York, United States
Introduction
TAFRO syndrome is a rare, life-threatening variant of idiopathic multicentric Castleman disease. Characterized by thrombocytopenia, anasarca, fever, renal dysfunction, and organomegaly, its heterogeneous presentation often mimics sepsis or malignancy, delaying diagnosis and treatment. Lymph node biopsy is the diagnostic gold standard, yet can often be histologically inconclusive.
In this case a kidney biopsy revealed critical histological clues when lymph node biopsy did not yeild definitive results. It highlights the importance of recognizing renal pathology as a diagnostic gateway for TAFRO syndrome, demonstrating that when other markers are ambiguous, the kidney can unmask this elusive disorder.
Case Description
A 22-year-old male presented with diffuse arthralgias. On workup, he was found to have anasarca and a constellation of severe anemia, thrombocytopenia, and AKI with proteinuria. Serologic testing for autoimmune, infectious, and paraproteinemic etiologies was negative. While CT imaging revealed hepatosplenomegaly and lymphadenopathy, a lymph node biopsy was non-revealing.
The patient’s arthralgias and AKI worsened. A kidney biopsy was done which demonstrated extensive mesangiolysis and glomerular endothelial injury (cell swelling and widening of the subendothelial GBM) consistent with early TMA. This led to the suspicion that the systemic inflammation and renal endothelial involvement were connected as a syndrome. IL-6 levels were obtained and found to be severely elevated. The constellation of symptoms, renal endothelial injury on biopsy, and elevated IL-6 strongly suggested TAFRO syndrome. The diagnosis was confirmed by bone marrow biopsy showing diffuse reticulin fibrosis. He was started on prednisone and transitioned to rituximab therapy.
Discussion
TAFRO syndrome is a rare, life-threatening systemic inflammatory disorder. Diagnosis can be delayed by its heterogeneous presentation and reliance on lymph node biopsy. While renal involvement is common, kidney histopathology—often revealing thrombotic microangiopathy (TMA) or membranoproliferative patterns—is underutilized as a diagnostic tool.
When traditional workup proved ambiguous, a kidney biopsy provided the evidence of endothelial injury, facilitating a timely TAFRO diagnosis. This case emphasizes that in clinically complex, heterogeneous presentations, renal biopsy can be the decisive diagnostic bridge to initiating life-saving immunosuppressive therapy