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Abstract: SA-PO0759

Proliferative Glomerulonephritis with Monoclonal Immunoglobulin Deposits Associated with Chronic Lymphocytic Leukemia with a Response to Clone-Directed Therapy: A Case Report

Session Information

Category: Glomerular Diseases

  • 1402 Glomerular Diseases: Clinical, Outcomes, and Therapeutics

Authors

  • Palad, Maria Karina, Lenox Hill Hospital, New York, New York, United States
  • Patel, Anuj A., Lenox Hill Hospital, New York, New York, United States
  • Rosenstock, Jordan L., Lenox Hill Hospital, New York, New York, United States
Introduction

Proliferative glomerulonephritis with monoclonal immunoglobulin deposits (PGNMID) is mostly not associated with a detectable clone. This is especially the case for the IgG3 subtype which is the most common. However, IgG1- variant PGNMID, which comprises 10-15% of cases, is more likely to be associated with an underlying hematologic disorder. We present a case of PGNMID in a patient with chronic lymphocytic leukemia (CLL) that showed good renal response after clone-directed therapy.

Case Description

This is an 84-year-old male with a history of CLL (not on treatment because the CLL was thought to be indolent), initially seen for rise in creatinine from 1.0mg/dL to 1.6mg/dL with proteinuria of 4.4g/g and microscopic hematuria. Renal biopsy showed PGNMID with IgG1 dominant deposits with lambda restriction and a membranoproliferative pattern. Bone marrow biopsy showed small leukemic B-cells co-expressing CD20+, CD5+, and CD23+ accounting for 10-15% of the total cellularity. Patient was started on prednisone, but creatinine continued to rise up to 4mg/dL. Given worsening renal function, treatment for CLL was initiated with a BTK inhibitor (acalabrutinib 100mg twice daily). After starting treatment, there was an improvement of kidney function to a creatinine of 1.9mg/dL and reduction in proteinuria to 1.4g/g (see figure).

Discussion

The findings support PGNMID secondary to CLL-associated immune dysregulation, that appeared to respond to clone-directed therapy. This case emphasizes the importance of recognition that PGNMID, particularly IgG1-variant, can be associated with an underlying hematologic neoplasia and treatment should be directed to the clone if identified, even if the underlying disorder was considered an indolent process.