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Abstract: FR-PO675

Immune Complex-Mediated Membranoproliferative Glomerulonephritis in TAFRO Syndrome: A Case Report

Session Information

Category: Glomerular Diseases

  • 1303 Glomerular Diseases: Clinical‚ Outcomes‚ and Trials

Authors

  • Hrizat, Alaa S., Thomas Jefferson University, Philadelphia, Pennsylvania, United States
  • Eberle-Singh, Jaime, Thomas Jefferson University, Philadelphia, Pennsylvania, United States
  • Li, Li, Thomas Jefferson University, Philadelphia, Pennsylvania, United States
Introduction

TAFRO syndrome is a clinical subset of idiopathic multicentric Castleman disease (iMCD) characterized by thrombocytopenia, anasarca, fever, reticulin fibrosis or renal dysfunction, and organomegaly. Renal involvement is a significant complication of TAFRO syndrome; however, only a small number of cases have been reported.

Case Description

A 42-year-old woman presented with hypertensive crisis and dyspnea. She was incidentally found to have a large mediastinal mass (11cm), pericardial and bilateral pleural effusions, and extensive multifocal hypermetabolic lymphadenopathy. The anterior mediastinal mass biopsy was diagnosed with iMCD with negative serologies for HIV and HHV-8. The patient was treated with 16 cycles of Siltuximab (anti-IL-6). While her dyspnea and metabolic activity of adenopathy improved, she continued to have anasarca and worsening hypergammaglobulinemia. This inflammatory state was consistent with TAFRO syndrome of iMCD and treatment was changed to Rituximab. Shortly after, the patient developed acute decompensated heart failure and worsening renal function with a Cr of 2.30 mg/dL (baseline Cr 1.5 mg/dL), BUN 65 mg/dL. Renal biopsy revealed that most of the glomeruli had membranoproliferative glomerulonephritis (MPGN) injury patterns without thrombi or fibrinoid necrosis. Immunofluorescence study revealed (all 1-2+) mesangial and capillary loop granular IgG, C3, kappa and lambda light chain deposits. Electron microscopy identified subendothelial electron-dense immune-type immune deposits. Overall the findings are consistent with immune deposit-associated MPGN.

Discussion

We present an iMCD patient with TAFRO syndrome with renal insufficiency secondary to immune deposit-mediated MPGN. Amyloidosis MPGN and thrombotic microangiopathy (TMA) have been reported in MCD. Further research is needed to elucidate the roles of VEGF and IL-6 and their impact on renal pathology in TAFRO syndrome.

A. Representative glomerulus shows MPGN injury pattern (H&E, 40X).