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Kidney Week

Abstract: SA-PO171

Monoclonal Immunoglobulin Tubulointerstitial Deposits in Kidney in Sjogren Syndrome with MALT Lymphoma: Occam's Razor or Hickam's Dictum

Session Information

  • Onco-Nephrology: Clinical
    November 09, 2019 | Location: Exhibit Hall, Walter E. Washington Convention Center
    Abstract Time: 10:00 AM - 12:00 PM

Category: Onco-Nephrology

  • 1500 Onco-Nephrology

Authors

  • Sharma, Purva D., Zucker School of Medicine at Hofstra/Northwell, Great Neck, New York, United States
  • Andrade paz, Hugo, Northwell Health, Great Neck, New York, United States
  • Bijol, Vanesa, Northwell Health Hofstra University, Lake Success, New York, United States
Introduction

Monoclonal gammopathy is a common phenomenon in patients with MALT ( Mucosa Associated Lymphoid Tissue) lymphoma likely due to clonal production of paraproteins by lymphoplasmacytic cells, which responds to B cell directed therapy. We report a case of Sjogren’s disease and MALT lymphoma with rearranged kappa light chain, serum monoclonal gammopathy, and monoclonal IgM-kappa tubulointerstitial deposits in the kidney, that presented with a challenging therapeutic dilemma

Case Description

60 yo woman with Sjogren’s disease presented with a subacute rise in creatinine from 1.4 to 1.8 mg/dl and 1 gm proteinuria. Work up was positive for low C3, undetectable C4, positive RF. SIFE showed weak IgM-kappa band, urine immunofixation showed kappa bence jones protein. Kappa/Lambda free light chain ratio was 23. She had a h/o parotid mass 2 years ago, diagnosed on excisional biopsy to be extra-nodal marginal zone lymphoma with IHC positive for CD20 and PCR showing rearranged kappa light chain. Repeat CT showed persistent low level + FDG activity in left eye and parotids that was decided to be monitored. A kidney biopsy was obtained that showed IgM kappa tubulointerstitial deposition disease with polytypic chronic active interstitial nephritis. Immunohistochemistry analysis revealed polytypic T cell predominant immunophenotype with no evidence of lymphoma. A subsequent work up included a bone marrow biopsy that showed a polytypic plasma cell population (0.6% of the cells) with a K/L ratio of 3:1. Immunohistochemistry showed polyclonal plasma cells. There was no evidence of myeloma or lymphoma. Considering the above, it was concluded that the low level lymphoma was likely the source of monoclonal protein and a decision was made to treat with B cell directed therapy only.

Discussion

Monoclonal gammopathy has been reported with MALT lymphomas but this is the first case in literature with associated monoclonal IgM-kappa tubulointerstitial deposition disease in the kidney. It is important to work up these patients for another clone producing site since it impacts decision making about immunosuppression. Close follow up is needed for monitoring renal and hematologic recovery.