Abstract: FR-PO1240
TAFRO Syndrome: A Diagnostic Challenge in a Patient with AKI and Anasarca
Session Information
- Onconephrology: Diagnostic Dilemmas, Therapy-Related Toxicities, and Clinical Cases
October 23, 2026 | Location: Exhibit Hall A, Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Onconephrology
- 1600 Onconephrology
Authors
- Sharara, Jana, Cleveland Clinic, Cleveland, Ohio, United States
- Kuperman, Michael Benjamin, Cleveland Clinic, Cleveland, Ohio, United States
- Gaba, Meenu, Cleveland Clinic, Cleveland, Ohio, United States
- Dhingra, Jagmeet S., Cleveland Clinic, Cleveland, Ohio, United States
Introduction
TAFRO syndrome, a subtype of HHV-8–negative idiopathic multicentric Castleman disease, is a life-threatening inflammatory disorder defined by thrombocytopenia, anasarca, fever, renal insufficiency, and organomegaly. Diagnosis is often delayed because early manifestations may mimic primary renal, cardiac, or hematologic disorders before characteristic features emerge.
Case Description
72-year-old woman with hypertension presented with progressive anasarca (recurrent pleural and trace pericardial effusions, ascites, and lower extremity edema), AKI (0.5>1.87 mg/dL), and proteinuria. Cardiorenal syndrome was initially suspected; however, echocardiography was normal to the degree of volume overload. Anasarca was also disproportionate to the transient proteinuria (UPCR >8 once, all subsequent values <2). Hematologic evaluation showed hemoglobin 10.5, platelets 113 without hemolysis. ADAMTS13 activity was 55%, not consistent with thrombotic thrombocytopenic purpura. Autoimmune and infectious studies were unrevealing, and monoclonal studies were indeterminate. Bone marrow biopsy showed nonspecific hypercellularity without evidence of myelodysplasia. Kidney biopsy demonstrated chronic thrombotic microangiopathy with mesangiolysis and endothelial injury. Despite extensive evaluation, no unifying diagnosis was identified. She was referred to our center and admitted for workup. Creatinine was 1.65 with UPCR 0.28. Elevated C reactive protein and erythrocyte sedimentation rate inflammatory markers prompted testing of IL-6 and VEGF, both elevated. PET/CT showed mild, non–FDG-avid lymphadenopathy. Excisional lymph node biopsy revealed regressed germinal centers, expanded mantle zones with concentric layering, vascular proliferation, and polytypic plasmacytosis, consistent with Castleman changes. Idiopathic multicentric Castleman disease (TAFRO subtype) was diagnosed. Treatment with Siltuximab resulted in marked clinical improvement and Cr recovery to 1.22.
Discussion
This case highlights the diagnostic challenge of TAFRO syndrome, which took a month-long hospitalization for definitive diagnosis. TMA-like findings in a kidney biopsy may reflect endothelial injury as part of a systemic inflammatory process rather than a primary renal disease. Diagnosis of a rare disease like TAFRO syndrome often requires evaluation of extrarenal tissue when renal findings do not explain the full the clinical presentation.