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      Type I Cryoglobulinemic Nephritis in a Patient of Monoclonal Gammopathy of Renal Significance

      case-report

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          Abstract

          Monoclonal gammopathy of renal significance is a recently described entity in which a small B-cell clone not meeting the criteria for the diagnosis of multiple myeloma produces renal disease usually through deposition of a secreted monoclonal immunoglobulin. Here, we describe a case of Type I cryoglobulinemic glomerulonephritis diagnosed on a kidney biopsy and caused by a monoclonal IgM produced by a small bone marrow clone. The patient made a complete renal recovery after chemotherapy to suppress the clone.

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          Most cited references7

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          C3 glomerulonephritis associated with monoclonal gammopathy: a case series.

          C3 glomerulonephritis (GN) is a proliferative GN resulting from glomerular deposition of complement factors due to dysregulation of the alternative pathway of complement. Dysregulation of the alternative pathway of complement may occur as a result of mutations or functional inhibition of complement-regulating proteins. Functional inhibition of the complement-regulating proteins may result from a monoclonal gammopathy. Case series. 32 Mayo Clinic patients with C3 GN, 10 (31%) of whom had evidence of a monoclonal immunoglobulin in serum. Clinical features, hematologic and bone marrow biopsy findings, kidney biopsy findings, kidney measures, complement pathway abnormalities, treatment, and follow-up of patients with C3 GN that was associated with a monoclonal gammopathy. Mean age of patients with C3 GN associated with monoclonal gammopathy was 54.5 years. Bone marrow biopsy done in 9 patients revealed monoclonal gammopathy of undetermined significance in 5 patients, small lymphocytic lymphoma/chronic lymphocytic leukemia in one patient, and no abnormal clones in the other 3 patients. Kidney biopsy showed membranoproliferative GN with bright capillary wall C3 staining in all 10 patients. Evaluation of the alternative pathway of complement showed abnormalities in 7 of 9 patients tested. No mutation in complement-regulating proteins was detected in any patient. As an index case, one patient with C3 GN and chronic lymphocytic leukemia was treated with rituximab, cyclophosphamide, vincristine, and prednisone, and one patient with C3 GN and monoclonal gammopathy of undetermined significance was treated with dexamethasone and bortezomib. Both patients showed significant decreases in hematuria and proteinuria and stabilization of kidney function. Studies to show evidence of direct activation of the alternative pathway by monoclonal immunoglobulin were not done. The study highlights the association of C3 GN and monoclonal gammopathy, in particular in the older population, and the importance of targeting the underlying hematologic malignancy as an approach to treating C3 GN. Copyright © 2013 National Kidney Foundation, Inc. Published by Elsevier Inc. All rights reserved.
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            Membranoproliferative glomerulonephritis secondary to monoclonal gammopathy.

            Membranoproliferative glomerulonephritis (MPGN) is an immune complex-mediated glomerulonephritis characterized by subendothelial and mesangial deposition of immune complexes. Autoimmune diseases and chronic infections, such as hepatitis C, are commonly recognized causes of MPGN; however, monoclonal gammopathy is a less widely recognized cause of MPGN. We reviewed all renal biopsies of MPGN in Mayo Clinic patients during a 6-year period to determine the association of monoclonal gammopathy with MPGN. Results were correlated with electrophoresis studies and bone marrow biopsies to clarify the relationship between MPGN and gammopathies. Of 126 patients with MPGN, 20 did not have workup for hepatitis B or C. Of the remaining 106 patients, 25 (23.5%) were positive for hepatitis B or C. Of the 81 hepatitis-negative patients, 13 were not evaluated for gammopathies. Of the remaining 68 patients, 28 (41.1%) had serum and/or urine electrophoresis studies positive for monoclonal gammopathy. Serum immunofixation electrophoresis was the most sensitive method for diagnosing monoclonal gammopathy. Renal biopsy showed a membranoproliferative pattern of injury; immunofluorescence microscopy was often instrumental in diagnosing the underlying gammopathy. On the basis of the bone marrow biopsy, monoclonal gammopathy of undetermined significance was the most common entity associated with MPGN. Other, less common causes included multiple myeloma, low-grade B cell lymphoma, and chronic lymphocytic leukemia. Monoclonal gammopathy is an important and common cause of MPGN; therefore, all patients with a diagnosis of MPGN should be evaluated for an underlying monoclonal gammopathy.
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              Dysproteinemia, proteinuria, and glomerulonephritis.

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                Author and article information

                Journal
                Indian J Nephrol
                Indian J Nephrol
                IJN
                Indian Journal of Nephrology
                Medknow Publications & Media Pvt Ltd (India )
                0971-4065
                1998-3662
                Jan-Feb 2018
                : 28
                : 1
                : 65-68
                Affiliations
                [1] Department of Nephrology, King Edward Memorial Hospital, Pune, Maharashtra, India
                [1 ] Department of Hematology, King Edward Memorial Hospital, Pune, Maharashtra, India
                [2 ] Department of Pathology, King Edward Memorial Hospital, Pune, Maharashtra, India
                Author notes
                Address for correspondence: Dr. V. A. Lobo, Department of Nephrology, King Edward Memorial Hospital, 489, Rasta Peth, Sardar Moodliar Road, Pune - 411 011, Maharashtra, India. E-mail: valentinelobo@ 123456rediffmail.com
                Article
                IJN-28-65
                10.4103/ijn.IJN_175_16
                5830812
                6a4ebdf5-de0c-4f9c-97bc-929609ad9d9f
                Copyright: © 2018 Indian Journal of Nephrology

                This is an open access article distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 3.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as the author is credited and the new creations are licensed under the identical terms.

                History
                Categories
                Case Report

                Nephrology
                cryoglobulinemia,light chain restriction,monoclonal gammopathy
                Nephrology
                cryoglobulinemia, light chain restriction, monoclonal gammopathy

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