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Abstract: PO1885

A Case of IgA Nephropathy in the Setting of Sezary Syndrome and Mogamulizumab

Session Information

Category: Onco-Nephrology

  • 1500 Onco-Nephrology

Authors

  • Doraiswamy, Mohankumar, The Ohio State University Wexner Medical Center, Columbus, Ohio, United States
  • Brodsky, Sergey V., The Ohio State University Wexner Medical Center, Columbus, Ohio, United States
  • Reneau, John C., The Ohio State University Comprehensive Cancer Center Arthur G James Cancer Hospital and Richard J Solove Research Institute, Columbus, Ohio, United States
  • Leisring, Joshua, The Ohio State University Wexner Medical Center, Columbus, Ohio, United States
Introduction

IgA Nephropathy (IgAN) is an autoimmune disease with complex pathogenesis. Sezary syndrome (SS) is a leukemic subtype of cutaneous T cell lymphoma (CTCL). A rare association has previously been reported between IgAN and CTCL. Mogamulizumab (MG) is a monoclonal antibody drug targeting C-C chemokine receptor type 4 (CCR4) and is used in the treatment of CTCL and SS. MG has been associated with drug eruptions and systemic immune-mediated adverse events.

Case Description

A 63 year-old woman with SS was treated with MG. Her skin symptoms improved and circulating Sezary cells cleared. Due to a cutaneous drug eruption, the frequency of MG administration was reduced to monthly after cycle 7. Labs prior to cycle 19 demonstrated serum creatinine (Cr) 1.77 mg/dL from a prior baseline ~0.9-1.0 mg/dL. She received intravenous fluids but Cr worsened to 3.97 mg/dL. Urinalysis (UA) revealed more than 20 red blood cells (RBCs) per high powered field (HPF). 24 hour urine protein to creatinine ratio (UPCR) was 2.03 g/g. Serologies and complements were normal except double stranded DNA which was 12 IU/mL (normal <4 IU/mL). Kidney biopsy demonstrated mesangial immune complex deposition with IgA, IgG, and C3 predominance consistent with IgAN (Oxford M1E0S1T1C0). Prednisone was initiated at 1 mg/kg/day and tapered over 6 months. MG was stopped. After 6 months Cr had improved to 1.10 mg/dL. UA showed 3-5 RBCs per HPF with UPCR of 0.122 g/g. Her SS remained well controlled without systemic therapy.

Discussion

This case reinforces the association between IgAN and CTCL which has been described in prior case series. In patients with CTCL, altered T cell populations and a dysregulated immune response may contribute to the pathogenesis of IgAN. Complicating this case is the use of MG which can deplete normal CCR4-expressing regulatory T cells by inducing antibody-dependent cellular cytotoxicity. MG is associated with cutaneous granulomatous drug eruptions in which alterations in T cell populations have been implicated. Systemic autoimmune complications outside of the kidneys have also been reported. The possibility that MG could play a role in IgAN should be considered.