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Abstract: TH-PO570

A Rare Feature of Myeloperoxidase (MPO)-Positive Crescentic Glomerulonephritis (GN) and Neural Epidermal Growth Factor-Like 1 (NELL-1) Protein Positive Membranous Nephropathy (MN) in Mantle Cell Lymphoma (MCL)

Session Information

Category: Glomerular Diseases

  • 1401 Glomerular Diseases: From Inflammation to Fibrosis

Authors

  • Mai, Erik, Medical University of South Carolina, Charleston, South Carolina, United States
  • ElSheikhMohammed, Waleed A., Medical University of South Carolina, Charleston, South Carolina, United States
  • Budisavljevic, Milos N., Medical University of South Carolina, Charleston, South Carolina, United States
  • Bruner, Evelyn, Medical University of South Carolina, Charleston, South Carolina, United States

Group or Team Name

  • MUSC Nephrology.
Introduction

MCL is a B-cell non-Hodgkin’s lymphoma that can have renal involvement via infiltration, immune complex (IC), or complement-mediated (CM) GN. Typical IC-positive GN lesions include proliferative GN with monoclonal IgG deposits (PGNMD), C3GN, and MN. Antineutrophilic Cytoplasmic Antibody (ANCA) associated GN is now recognized to link with preceding or concurrent malignancy. Our case features a rare presentation of acute kidney injury (AKI) caused by MCL infiltration, coexisting MPO-ANCA associated crescentic GN, and NELL-1 positive MN.

Case Description

61-year-old previously healthy male presented with intermittent fever, night sweats, maculopapular rash with bullae, anasarca and a 30 lbs weight loss. He was hypertensive and labs revealed anemia, thrombocytopenia, Strep. Agalactiae bacteremia, active urine sediment and severe AKI needing dialysis. Also notable was hypoalbuminemia 1.9 g/dL with proteinuria 9 g/g. Imaging revealed splenomegaly, diffuse lymphadenopathy, and normal-sized kidneys. Nephritic labs showed positive ANCA with MPO antibody specificity, low C3 and C4 levels, and normal kappa lambda light chain ratio. Bone marrow biopsy confirmed MCL with blastoid features. Renal biopsy showed crescentic GN, polyclonal IgG, C3, C1q, Kappa and Lambda along the tubular basement membrane, positive NELL-1 staining, and lymphomatous infiltration. Steroids and Rituximab were used as induction therapy without plasmapheresis. Rituximab continued as part of MCL chemotherapy. His renal function improved and dialysis was stopped. At 8 months creatinine was 2.9 g/dl with partial nephrosis remission (UPCR 1.3 g/g and albumin 3.3 g/dL).

Discussion

We describe a rare AKI in lymphoma presentation with MCL infiltration, MPO positive crescentic GN, and NELL-1 associated MN. To our knowledge no such case has been reported. The association between NELL-1 and malignancy is established, but it's unclear if this association with MPO ANCA is coincidental. Early identification through kidney biopsy and initiation of cancer treatment may alter the renal and patient overall prognosis. Our patient responded well to Rituximab and steroids, but the success of standard guideline therapy and the possibility of complete remission require further investigation.