THE CHARACTERISTICS OF PROLIFERATIVE GLOMERULONEPHRITIS WITH MONOCLONAL IMMUNOGLOBULIN DEPOSITS OF IgG2 SUBCLASS

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THE CHARACTERISTICS OF PROLIFERATIVE GLOMERULONEPHRITIS WITH MONOCLONAL IMMUNOGLOBULIN DEPOSITS OF IgG2 SUBCLASS

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Co-author 1

Lihong Bu bu.lihong@mayo.edu Mayo Clinic Rochester Department of Laboratory Medicine and Pathology Rochester United States *

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Samih Nasr nasr.samih@mayo.edu Mayo Clinic Rochester Department of Laboratory Medicine and Pathology Rochester United States -

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Introduction

Most cases of proliferative glomerulonephritis with monoclonal immunoglobulin deposits (PGNMID) are associated with IgG3. PGNMID-IgG2 is very rare and its clinical pathologic characteristics have not been studied.

Methods

We retrospectively identified 13 PGNMID-IgG2 cases from our nephropathology archives. PGNMID-IgG2 was defined by proliferative glomerulonephritis with glomerular granular monotypic IgG2 deposits after excluding cryoglobulinemic glomerulonephritis.

Results

The patients were 62% male with a median age of 58 years who presented with proteinuria (median 3.5 g/day), hematuria (62%), and renal insufficiency (median serum creatinine 1.6 mg/dL). Hypocomplementemia was uncommon (11%). Four (31%) patients had low-grade B-cell lymphoma, including 3 CLL/SLL and 1 marginal zone lymphoma. SPEP/SIF detected the nephropathic MIg in 25%, and bone marrow biopsy detected clonal B-cells in 1 patient and clonal plasma cells in 2 patients, both less than 10%. Kidney biopsy revealed mesangial proliferative (54%) pattern and MPGN (38%), with membranous features (38%), non-organized glomerular monotypic IgG2 (100%), C3 deposition (100%), and C1q deposition (31%).

Conclusion

PGNMID-IgG2 mostly affects middle-aged patients and presents with heavy proteinuria and renal dysfunction. It is more commonly associated with B-cell clones (particularly CLL/SLL, present in a quarter of patients) than plasma cell clones. Larger studies are needed to study the optimal treatment for this disease.

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