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Kidney Week

Abstract: SA-PO0761

Rapidly Progressive Cryoglobulinemic Glomerulonephritis in Waldenstrom Macroglobulinemia: When the Kidney Can't Wait

Session Information

Category: Glomerular Diseases

  • 1402 Glomerular Diseases: Clinical, Outcomes, and Therapeutics

Authors

  • Yousaf, Uzma, Loyola University Chicago, Chicago, Illinois, United States
  • Iftikhar, Muhammad Ali, Loyola University Chicago, Chicago, Illinois, United States
  • Ling, Benjamin, Loyola University Chicago, Chicago, Illinois, United States
Introduction

Renal involvement in Waldenstrom macroglobulinemia (WM) is uncommon, occurring in <5% of patients. WM can lead to a broad clinicopathological spectrum of paraprotein-mediated renal injury, including amyloidosis, cast nephropathy, and rare types of monoclonal gammopathy of renal significance (MGRS), such as cryoglobulinemic glomerulonephritis (cryo-GN). We present a case of rapidly progressive cryo-GN leading to dialysis despite BTK-inhibitor therapy (Zanubrutinib).

Case Description

A 70-year-old Caucasian male with a history of brachytherapy-treated prostate cancer and a recent diagnosis of Waldenstrom macroglobulinemia/ Lymphoplasmacytic lymphoma (WM/LPL) on Zanubrutinib, presented with frailty, severe weight loss, hypotension, and acute kidney injury (AKI) initially thought to be of pre-renal etiology. His course was complicated by a progressive decline in his renal function that was associated with persistent hypotension and malnutrition. Workup was notable for persistent hematuria, proteinuria (ACR 711 mg/g, PCR 2.4 g/g), low C3/C4, abnormal K:L ratio, and elevated IgM levels, which raised concern for underlying MGRS. Ultimately, a kidney biopsy was performed, showing cryo-GN with monoclonal IgM-Keppa-related disease. As his renal dysfunction progressed to dialysis dependence, his hematologist initiated plasmapheresis and escalated his chemoimmunotherapy with Bendamustine-Rituximab(BR) in hopes of renal recovery with rapid debulking of cryoglobulins.

Discussion

This case highlights a poor hematological response to BTK inhibitors contributing to a concordant development of cryoglobulin-mediated renal injury. Due to the absence of overt systemic vasculitis features, a renal biopsy was necessary to discover this rare complication of WM-related cryoglobulin-mediated MPGN.