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Kidney Week

Abstract: SA-PO193

No More Wandering To and Fro: Shedding Light on TAFRO

Session Information

Category: Onconephrology

  • 1700 Onconephrology

Authors

  • Gandhi, Nisarg, Stanford Medicine, Stanford, California, United States
  • Zhao, Xixi, VA Palo Alto Health Care System, Palo Alto, California, United States
Introduction

TAFRO syndrome is a systemic inflammatory disease characterized by thrombocytopenia (T), anasarca (A), fever (F), reticulin fibrosis (R), and organomegaly (O), often accompanied by AKI. Recently described in 2010 through a case series of Japanese patients, TAFRO is now recognized as a subtype of human-herpesvirus 8 (HHV8) negative idiopathic multicentric Castleman disease (iMCD). Herein, we present a rare case of TAFRO syndrome with AKI requiring dialysis, who was successfully treated with rituximab-based chemotherapy.

Case Description

A 45-year-old previously healthy male presented with anasarca and was found to have new thrombocytopenia, leukocytosis, anemia, and extensive abdominal and axillary lymphadenopathy. After undergoing an excisional lymph node biopsy and a bone marrow biopsy, he was diagnosed with HHV8-negative iMCD, TAFRO subtype, and started on dexamethasone and siltuximab. Despite treatment, creatinine rose from 0.8 mg/dL to 3.11 mg/dL with hyperkalemia requiring hemodialysis. Labs were notable for IL-6 of 35.6 pg/mL (ref: <5 pg/mL) and VEGF of 446 pg/mL (ref: 31-86 pg/mL). UA was negative for proteinuria and hematuria. Renal biopsy showed intact glomerulus with mesangiolysis, glomerular basement membrane double contouring, and scattered fragmented RBCs consistent with thrombotic microangiopathy (TMA). Given severe features of the disease without improvement in renal function, he was initiated on a rituximab-based chemotherapy regimen (R-CHOP) with immediate response. His kidney function returned to its previous baseline two weeks later.

Discussion

TAFRO syndrome is an underrecognized condition that can result in AKI requiring dialysis. Despite the majority of TAFRO cases describing AKI as a central feature, descriptions of renal histology and treatment regimens have been limited in the literature. Prior kidney biopsies have commonly reported MPGN and TMA, which led to the postulated mechanism of injury to occur via IL-6 and VEGF leading to glomerular microangiopathy. Our patient was refractory to IL-6 blockade with siltuximab, suggesting the pleomorphic cytokine profile of iMCD and that IL-6 may not be the primary driver of inflammation. Due to the rarity of the condition and paucity of data guiding treatment, our case adds to the otherwise limited clinical description, renal histology, and treatment of TAFRO syndrome.