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Kidney Week

Abstract: PO0284

An Unusual Presentation of Type 1 Cryoglobulinemic GN in Monoclonal Gammopathy of Undetermined Significance (MGUS)

Session Information

Category: Acute Kidney Injury

  • 102 AKI: Clinical, Outcomes, and Trials

Authors

  • Han, Heedeok, Columbia University Irving Medical Center, New York, New York, United States
  • Zachariah, Teena, Columbia University Irving Medical Center, New York, New York, United States
  • Rosen, Raphael Judah, Columbia University Irving Medical Center, New York, New York, United States
  • Santoriello, Dominick, Columbia University Irving Medical Center, New York, New York, United States
Introduction

Type I cryoglobulinemia can develop in the setting of monoclonal gammopathy of undetermined significance (MGUS) and can have renal involvement in a third of cases. We present an unusual case of Type I cryoglobulinemic glomerulonephritis (GN).

Case Description

An 83-year-old woman with a history of IgG Kappa MGUS presented with decreased urine output and nausea. During the previous six months, the patient was hospitalized three times for acute kidney injury (AKI) requiring dialysis. Two renal biopsies had been performed during those admissions, which showed GN with scant immune deposit. One glomerulus had intraluminal staining for IgG and Kappa suggesting Type 1 Cryoglobulinemic GN secondary to her MGUS (Figure 1, A-C). She declined chemotherapy at the time, however, each time her renal function improved spontaneously and she was discharged without requiring dialysis.

In the ED, her blood pressure was 182/69 but her vitals were otherwise normal. The physical exam was unremarkable. Labs were notable for sodium 122 meq/L, potassium 5.2 meq/L, creatinine 4.5 mg/dL, albumin 3.4 g/dL, low C3 and C4, and positive MPO-ANCA. The urinalysis showed 50 red blood cells, 15 white blood cells, and random urine protein > 2000mg/dL. Other serologic and infectious labs, including serum cryoglobulin, were negative. The renal ultrasound was normal. The patient’s renal function worsened and was started on dialysis. This time the patient agreed to chemotherapy and immunosuppression with the aim to prevent further recurrences of AKI.

The patient was started on clone-directed therapy with cyclophosphamide, bortezomib, and dexamethasone. Plasma exchange therapy was also performed for the clearance of light chains. The patient’s renal function improved and she was discharged without requiring dialysis. Her two-month follow up creatinine was 1.08mg/dL.

Discussion

This case of type 1 cryoglobulinemic GN is unusual in that the patient developed cyclical but self-resolving episodes of AKI-D that was successfully treated with clone-directed therapy.

Figure 1.
A. Immune Thrombi on H&E
B, C. IgG and Kappa staining on Immunofluorescence