Abstract: SA-PO228
Type I Cryoglobulinemic Glomerulonephritis in Solitary Kidney
Session Information
- Onconephrology: Immunological Cross-Talk
November 04, 2023 | Location: Exhibit Hall, Pennsylvania Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Onconephrology
- 1700 Onconephrology
Authors
- Morris, Austin M., Corewell Health, Royal Oak, Michigan, United States
- Jacobs, Matthew, Corewell Health, Royal Oak, Michigan, United States
- Abu-Khaled, Jamal, Corewell Health, Royal Oak, Michigan, United States
Introduction
Cryoglobulinemia (CG) is a disease in which immunoglobulins precipitate in serum at lower temperatures and then redissolve once warmed again. Glomerulonephritis (GN) is a common complication with type II and III CG but rarely documented with type I. We describe a patient with type I CG (IgM) induced GN without identified source.
Case Description
66-year-old male with past medical history of a. fib, OSA, and solitary right kidney s/p nephrectomy at age 8 presenting with lower extremity edema and dyspnea on exertion found to be in hypertensive crisis. He denied hematuria or dysuria. Initial blood pressure of 212/104, started on nitroglycerin. Urinalysis with 2+ blood, >20 RBC, >500 protein, and granular casts. BMP electrolytes normal, BUN 37 mg/dL, creatinine 1.63 mg/dL (baseline 1.02). Normal TSH, metanephrines, and cortisol levels. Hepatitis panel negative. Negative renal arterial doppler. Nephritic workup with low C3, normal C4, and nephrotic range proteinuria (5.41 grams). SPEP with IgM monoclonal protein. Bone marrow biopsy negative, peripheral cytology with monoclonal B-cell population (CD5+, CD23-), cytogenetics negative. Renal biopsy IF studies IgM 3+, IgG 1-2+, C3 1-2+, and lambda 1+. EM with membranoproliferative pattern, focal hyalin thrombus, segmental fusion of foot processes, paired tubular structures identified in subendothelial spaces and pseudo-thrombus consistent with cryoglobulinemic GN. No subepithelial deposits. R-CHOP started for suspected lymphoma. Developed vasculitic lesions in mid-2021, C3 and C4 low, cryocrit positive for the first time. Transitioned to bendamustine with plasmapheresis. Second opinion felt lymphoma unlikely and started Velcade. Third opinion recommended ibrutinib with plasmapheresis for 4 weeks, renal function remains stable, blood pressure controlled but IgM band on serum immunofixation and urine sediment remain active.
Discussion
GN is common in mixed CG but extremely rare in type I, with our literature review showing 11 cases. Of those, 9 were due to hematolymphoid disease and the remaining 2 are IgG monoclonal bands. Our case shows that type I IgM CG can present with renal involvement and should be evaluated for in patients with unexplained AKI and positive IgM monoclonal protein with kidney biopsy. Furthermore, this case is unique in showing that renal biopsy can be done safely in a solitary kidney in order to have an accurate diagnosis.