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Kidney Week

Abstract: SA-PO204

A Rare Case of Proliferative Glomerulonephritis with Monoclonal IgA Lambda Deposits with Crescents

Session Information

Category: Onconephrology

  • 1700 Onconephrology

Authors

  • Mareedu, Neeharik, UPMC Western Maryland, Cumberland, Maryland, United States
  • Pilla, Ravi Teja, Virginia Commonwealth University Health System, Richmond, Virginia, United States
Introduction

Proliferative glomerulonephritis with monoclonal immunoglobulin deposits (PGNMID) is an uncommon condition under the spectrum of renal disorders grouped under monoclonal gammopathies with renal significance (MGRS). Even more uncommon is the type of immunoglobulin (Ig) deposition other than IgG subclasses (IgG3 heavy chain being most common type of deposition) in PGNMID, which may be IgA or IgM as well as light chain only deposition.

Case Description

We report a 65 y/o Caucasian female patient (pt) with history of hypertension, recurrent urinary tract infections and non-obstructive nephrolithiasis with recent worsening of creatinine (Cr) to 1.3-1.6 mg/dL, with albuminuria 1.8 g/g, proteinuria 2.2 g/g and hematuria and negative nephritic workup. Pt was also noted to have low kappa/lambda light chain ratio (0.14) with significantly elevated free lambda light chains (206.23). No significant abnormalities were noted on serum protein electrophoresis, serum immunofixation electrophoresis or urine protein electrophoresis. While awaiting hematology consultation and renal biopsy the pt was admitted with low back pain and was noted to have new retroperitoneal mass/adenopathy concerning lymphoma. Pt developed progressive renal failure necessitating initiation of hemodialysis (HD). Retroperitoneal mass biopsy positive for diffuse large B-cell lymphoma (DLBCL) with renal biopsy notable for proliferative glomerulonephritis with monoclonal IgA lambda deposition with crescents in the setting of lymphoma and MGRS w/ monoclonal IgA lambda. The pt was started on R-CHOP chemotherapy (CT) for management of her DLBCL while continuing her on HD. Pt eventually achieved DLBCL remission as well as renal recovery with discontinuation of HD with 2 R-CHOP and 4 R-COEP cycles.

Discussion

PGNMID is a rare cause of renal disorder. Though it is usually recognized with deposition of IgG3 subclass heavy chains in glomeruli, it can rarely be recognized with deposition of other immunoglobulins like IgA and IgM as well as light chains. Treatment of this disease is based on clone directed therapy, which could lead to complete or partial remission in these patients. Our case is a one of the rarer cases as it involved uncommon monoclonal IgA lambda chain deposition and it is also associated with diffuse large B-Cell lymphoma.