Abstract: FR-PO143
Successful Management of TAFRO Syndrome-Related Renal Thrombotic Microangiopathy with Interleukin-6 Inhibitor
Session Information
- AKI: Outcomes, RRT
November 03, 2023 | Location: Exhibit Hall, Pennsylvania Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Acute Kidney Injury
- 102 AKI: Clinical, Outcomes, and Trials
Authors
- Abdelkader, Ahmed I. Kamal, Medical University of South Carolina, Charleston, South Carolina, United States
- Vilayet, Salem, Medical University of South Carolina, Charleston, South Carolina, United States
- Bruner, Evelyn, Medical University of South Carolina, Charleston, South Carolina, United States
- Achanti, Anand, Medical University of South Carolina, Charleston, South Carolina, United States
- Budisavljevic, Milos N., Medical University of South Carolina, Charleston, South Carolina, United States
Introduction
TAFRO [Thrombocytopenia, Anasarca (edema, pleural effusions, ascites), Fever, Reticulin myelofibrosisis (or Renal insufficiency), and Organomegaly (lymphadenopathy and hepatosplenomegaly)] that represents a severe subtype of idiopathic multicentric Castleman’s disease (iMCD). Kidney involvement in TAFRO syndrome can be severe and may require hemodialysis (HD). However, detailed descriptions of this syndrome's aspect remain limited.
Case Description
A 20-year-old male presented as transfer from another hospital with anasarca, malaise, diffuse lymphadenopathy, and non-oliguric acute kidney injury. He has no past medical history. Initial concern for lymphoma; however, bone marrow biopsy, ascites, pleural fluid examination and flow cytometry were negative for malignancy. The lymph node biopsy showed diagnostic findings of Castleman disease-TAFRO variant. HHV-8, HHV-6, CMV, EBV and HIV were negative. Kidney functions continued to deteriorate driving hyperkalemia necessitated HD. Kidney biopsy was done showing thrombotic microangiopathy (TMA) with mesangiolysis. Serum Interleukin-6 (IL-6) and VEGF were high. Prednisolone was started and Siltuximab, an IL-6 monoclonal antibody, was initiated and continued at 3 weeks intervals. Patient started to improve within days after the first dose of IL-6 inhibitor and HD was discontinued along with full improvement of clinical symptoms and kidney functions.
Discussion
Rates of renal failure are much higher in TAFRO syndrome variant than typical iMCD. Histopathology of renal involvement in TAFRO syndrome has been reported in few publications. The majority have demonstrated a pattern consistent with membranoproliferative glomerulonephritis and few showed TMA. iMCD is known as hyper-IL-6 syndrome with polyclonal lymphadenopathy. Early recognition of disease and early initiation of IL-6 inhibition and glucocorticoids are of paramount importance in attaining complete.