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Abstract: PO1901

NELL-1 Membranous Nephropathy Associated with Diffuse Reactive Lymphadenopathy

Session Information

Category: Onco-Nephrology

  • 1500 Onco-Nephrology

Authors

  • Elabd, Hatem, TriHealth, Cincinnati, Ohio, United States
  • Murphy, Joel D., Arkana Laboratories, Little Rock, Arkansas, United States
  • Rashid, Tarek, Bayonne Medical Center, Bayonne, New Jersey, United States
  • Sawaf, Hanny, Cleveland Clinic, Cleveland, Ohio, United States
Introduction

We present a case of neural epidermal growth factor like-1 (NELL-1) MN associated with diffuse lymphadenopathy without evidence of malignancy or autoimmune disease.

Case Description

A 53-year-old woman with a BRCA 1 mutation presented with nephrotic syndrome. Diffuse lymphadenopathy was found on examination. Laboratory evaluation revealed serum albumin of 1.9 mg/dl, creatinine of 0.4 mg/dl, and UPCR 13.5 g/g. Urine microscopy showed protein (4+), and bland urine sediment. Serum PLA2R antibody was negative. Four excisional lymph node biopsies were performed, all of which revealed reactive hyperplasia without evidence of hematological malignancy. Flow cytometry and a bone marrow biopsy were negative. Serology for ANA, CRP, ESR, C3, C4, EBV, HIV, and Hepatitis B, and C were all negative. Renal pathology revealed diffuse, fine pinholes along the glomerular basement membranes using a Jones sliver stain. By immunofluorescence (IF), glomeruli showed diffuse, global finely granular capillary loop staining for IgG (3+), C3 (1-2+), and stained equally for κ and λ light chains. An IF stain for NELL-1 showed diffuse granular staining capillary loops (3+) while immunohistochemical stains for PLA2R and EXT2 were essentially negative. The ultrastructural evaluation revealed numerous, confluent subepithelial electron-dense deposits along with severe foot process effacement.

Discussion

NELL-1 associated MN was recently discovered as a distinct type of MN. In one series, 33 % of NELL-1 MN associated with malignancies, which is more often than the other known types of MN. The constellation of findings in this case with NELL-1 MN associated with reactive diffuse lymphadenopathy without evidence of malignancy or autoimmune disease is a rare presentation. Therefore, this case adds to the existing literature on NELL-1 associated MN, which helps to raise the awareness of this novel clinical presentation.

NELL-1 granular capillary loops staining