Abstract: TH-PO949
Minimally Proliferative Glomerulonephritis with Mesangial Deposition of Monoclonal IgM Lambda in a Patient with Marginal Zone Lymphoma and Acute Hepatitis C
Session Information
- Glomerular Trainee Case Reports
November 07, 2019 | Location: Exhibit Hall, Walter E. Washington Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Trainee Case Report
- 1202 Glomerular Diseases: Immunology and Inflammation
Authors
- Ghimire, Sudip, Dartmouth Hitchcock Medical Center, Hanover, New Hampshire, United States
- Pettus, Jason R., Dartmouth-Hitchcock Medical Center, Lebanon, New Hampshire, United States
- Graber, Martha L., Dartmouth Hitchcock Medical Center, Hanover, New Hampshire, United States
Introduction
We present an unusual renal manifestation of splenic marginal cell lymphoma and hepatitis C virus infection.
Case Description
A 57 year-old man presented with recurrent angio-edema. Abdominal CT showed splenomegaly and enlarged lymph nodes. Bone marrow biopsy showed splenic marginal cell lymphoma (MCL) with monoclonal lambda restriction.
Three weeks later he developed acute hepatitis C, viral load > 30 x 106 / IU/ml . One month later he developed nephrotic syndrome and RBC casts. Urine protein increased from 14 mg/dl to 7.8 g/day, serum albumin 3.2 g/dl. Serum cryoglobulins were negative and creatinine was 1.0 ml/dl. ANA, ANCA, C3, C4 were normal.
Renal biopsy showed mild focal mesangial hypercellularity and no endocapillary proliferation, necrotizing lesions, crescents, or microthrombi. Congo red negative. There was granular mesangial deposition of IgM lambda. IgG, C3, IgA, Kappa, and C1q were negative. EM showed diffuse foot process effacement and no fibrillary or microtubular forms.
Discussion
MCL is rare, 5-10% of NHL. There is an association of MCL with HCV seropositivity, mechanism unknown. Case reports describe 3 patients with MCL and MPGN with monoclonal IgM deposits, either IgM kappa or IgM lambda predominant. Our patient had acute HCV with negative cryoglobulins; previous cases had both chronic HCV infection and cryoglobulinemia or cryoglobulinuria. Electron microscopy in our patient did not show cryoglobulinemic immune deposits. The hepatitis C virus may be causally involved in MPGN with monoclonal IgM associated with MCL by a mechanism independent of the production of cryoglobulins.
References
1 Chelioti E, Efthimiou E et al Nephrourol Mon 2014 (Jul; 6 (4) e18391
2 Yamada M, Deitzer, D et al Am J KidneyDisease2016; 67 (5) A1-A118
3 Bracci, P, Benavente, Y et al J Natl Cancer Inst monographs 2014 48, 52-66;
IgM Lambda in Mesangium