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Kidney Week

Abstract: PO2196

A Case of Mixed Cryoglobulinemia Type II with Monoclonal Gammopathy in a Patient with Chronic Hepatitis B

Session Information

  • Onco-Nephrology - 2
    October 22, 2020 | Location: On-Demand
    Abstract Time: 10:00 AM - 12:00 PM

Category: Onco-Nephrology

  • 1500 Onco-Nephrology

Authors

  • Tan, Hui Zhuan, Singapore General Hospital, Singapore, Singapore
  • Nagarajan, Chandramouli, Singapore General Hospital, Singapore, Singapore
  • Loh, Alwin Hwai Liang, Singapore General Hospital, Singapore, Singapore
  • Koniman, Riece, Singapore General Hospital, Singapore, Singapore
  • Choo Chon Jun, Jason, Singapore General Hospital, Singapore, Singapore
Introduction

Non-Hepatitis C related mixed cryoglobulinemia Type II (MCT2) is rare. Causes include lymphoproliferative disorders, chronic infections like Hepatitis B and autoimmunity.

Case Description

A 68-year-old Chinese male presented with livedo reticularis and rapidly progressive glomerulonephritis requiring dialysis, on a background of chronic Hepatitis B (HBV) infection. Investigations showed a uPCR of 9.75g/g and serum creatinine (sCr) of 407 µmol/L (baseline sCr 82µmol/L). Rheumatoid factor was positive (>650U/ML). Both C3/C4 were low. HBV viral load was detected at 479, 452 copies/ml, while HCV, HIV and autoimmune screen were negative. A IgM kappa monoclonal band (3g/L) was detected on serum protein electrophoresis and immunofixation. Renal biopsy revealed MPGN with 28% crescents, capillary luminal hyaline thrombi, and IgM Kappa deposits. Findings were consistent with MCT2 despite negative serum cryoglobulins. Bone marrow aspiration and biopsy revealed a small clonal B-cell population confirmed on flow cytometry. Pulsed methylprednisolone followed by high-dose prednisolone, Rituximab and plasmapheresis were initiated for organ-threatening disease, together with entecavir. HBV viral suppression was observed after 1 month. Partial remission was achieved (sCr 140µmol/L; uPCR 2.39g/g) after 4 months. Development of sepsis and CMV reactivation prompted withdrawal of steroids, resulting in progressive deterioration of renal function (sCr 302µmol/L; uPCR 10.62 g/g). Commencement of Bortezomib and dexamethasone resulted in gradual improvement of renal function (sCr 107µmol/L; uPCR 4.71 g/g) after 3 cycles.

Discussion

MCT2 observed in our patient was possibly contributed by chronic HBV. However, suboptimal response despite HBV viral suppression prompted further treatment of the underlying lymphoproliferative disease causing monoclonal gammopathy of renal significance. Optimal treatment of MCT2 is not established, although immunosuppression consisting of corticosteroids with Rituximab or cyclophosphamide is required in severe renal involvement. We demonstrated a favourable outcome with Bortezomib-based clone directed therapy after achieving a non-sustained response with Rituximab/prednisolone. Long-term monitoring of the underlying low grade non-Hodgkin's lymphoma (NHL) is necessary.