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Kidney Week

Abstract: TH-PO565

Type II Cryoglobulinemic Glomerulonephritis in a Patient with Marginal Zone Lymphoma

Session Information

Category: Glomerular Diseases

  • 1401 Glomerular Diseases: From Inflammation to Fibrosis

Authors

  • Sivakumar, Devyani, Brown University, Providence, Rhode Island, United States
  • Shah, Senin, Brown University, Providence, Rhode Island, United States
  • Vishwanath, Manini, Brown University, Providence, Rhode Island, United States
Introduction


Cryoglobulinemia is rare and clinically significant in about 1 in 100,000. Renal involvement is seen in 30-60% cases and is especially associated with type II cryoglobulinemia. In a study of 80 patients with noninfectious biopsy proven mixed cryoglobulinemic glomerulonephritis, 7 patients had marginal zone lymphoma.

Case Description


A 76-year-old female with history of marginal zone lymphoma since 2019, managed conservatively, presented to the hospital with dyspnea on exertion, fatigue, 20 lbs weight gain and leg edema for 2 months. She had 3 recent hospitalizations for similar complaints and had been managed with diuretics and antibiotics, with no improvement. She was hypertensive to 167/86 mmHg, exam showed bilateral cervical lymphadenopathy and 2+ pitting pedal edema up to the groin. Labs revealed Cr 1.51 mg/dl [baseline 0.6-0.9 mg/dl]. UA positive for protein, albumin 2.4 g/dl and UPCR 9 g. Low complements with C3 at 33 mg/dl and C4 <3 mg/dl. Serology revealed ANA reactive [1:5120] and anti dsDNA negative. HIV, Hepatitis B and C were non-reactive. Kappa/Lambda ratio 1.68 with IF positive for monoclonal IgM kappa and potential monoclonal lambda free light chain. Immunoglobulin levels notable for elevated IgM at 1349 mg/dl. Cryoglobulin positive at 790 UL cryo/mL with isotype positive for weak monoclonal IgM kappa. Kidney biopsy showed membranoproliferative glomerular injury pattern with immune complexes which stained for IgM [3+], IgG [2+], C3 [2+], C1q [2+], kappa [2+], lambda light chains [1+]. A diagnosis of paraneoplastic type II cryoglobulinemia related to marginal zone lymphoma was made. She started treatment with rituximab, cyclophosphamide and dexamethasone and responded well with improvement in symptoms and renal function.

Discussion


Noninfectious type II cryoglobulinemic glomerulonephritis associated with marginal zone lymphoma is uncommon. In a retrospective multicenter survey of 242 cases of noninfectious mixed cryoglobulinemic vasculitis, 22 cases had marginal zone lymphoma and renal involvement was seen in 84 cases. Management and prognosis is dependent on disease severity and involves treatment of underlying cancer. Although our patient responded well to treatment, there was a 2 month delay in diagnosis despite recurrent hospitalizations. It is important to highlight this association and the clinical presentation.