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      Non Hodgkin's lymphoma associated membranoproliferative glomerulonephritis: rare case of long term remission with chemotherapy: a case report

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      Cases Journal
      BioMed Central

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          Abstract

          Introduction

          Although membranoproliferative glomerulonephritis has been reported to occur in association with non-Hodgkin's lymphoma, information concerning the long term effects of treatment of non-Hodgkin's lymphoma on the associated membranoproliferative glomerulonephritis is limited.

          Case presentation

          The current report describes a patient who presented with the abrupt onset of hypertension, mixed nephritic/nephrotic syndrome and acute renal failure. Kidney biopsy was consistent with membranoproliferative glomerulonephritis, type 1. Bone marrow biopsy performed in the evaluation of periaortic lymphadenopathy, hepatosplenomegaly, and thrombocytopenia confirmed the diagnosis of low grade B-cell non-Hodgkin's lymphoma. The patient's renal function improved and proteinuria resolved after initial treatment of non-Hodgkin's lymphoma with chemotherapy. During eleven years of follow up, membranoproliferative glomerulonephritis has remained in remission, as confirmed by repeatedly negative urinalyses, normal blood pressure and absence of clinical signs and symptoms suggestive of nephritic/nephrotic syndrome.

          Conclusion

          Membranoproliferative glomerulonephritis has been known to be associated with both chronic lymphocytic leukemia and non-Hodgkin's lymphoma, particularly with B cell lymphocytic type non-Hodgkin's lymphoma. There is limited information available concerning the effects of treatment of non-Hodgkin's lymphoma on the progression of non-Hodgkin's lymphoma associated membranoproliferative glomerulonephritis. In the few reported cases we found, long term follow up after initial resolution of the membranoproliferative glomerulonephritis was lacking. This report presented a rare case of non-Hodgkin's lymphoma associated membranoproliferative glomerulonephritis, that continued to be in remission during eleven years of follow up after initial chemotherapy treatment of lymphoma.

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          Most cited references10

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          Proliferative glomerulonephritis with monoclonal IgG deposits: a distinct entity mimicking immune-complex glomerulonephritis.

          Renal disease related to the deposition of monoclonal immunoglobulins containing both heavy and light chains can occur in type 1 cryoglobulinemia, Randall type light and heavy chain deposition disease (LHCDD), and immunotactoid glomerulonephritis. We report a novel phenotype of glomerular injury that does not conform to any of the previously described patterns of glomerular involvement by monoclonal gammopathy. Ten cases of unclassifiable proliferative glomerulonephritis manifesting glomerular monoclonal immunoglobulin G (IgG) deposits were identified retrospectively from the archives of the Renal Pathology Laboratory of Columbia University over the past 3 years (biopsy incidence 0.21%). The monoclonal immunoglobulins formed granular electron dense deposits in mesangial, subendothelial, and subepithelial sites, mimicking ordinary immune complex-mediated glomerulonephritis and producing a diffuse endocapillary proliferative or membranoproliferative glomerulonephritis. However, by immunofluorescence, the deposits were monoclonal, staining for a single light chain isotype and a single gamma subclass (including two IgG1kappa, one IgG1lambda, one IgG2lambda, four IgG3kappa, and one IgG3lambda). All cases stained for the three constant domains of the gamma heavy chain (CH1, CH2, and CH3), suggesting deposition of a nondeleted immunoglobulin molecule. Tissue fixation of complement was observed in 90% of cases, and 40% of patients had hypocomplementemia. Clinical presentations included renal insufficiency in 80% (mean serum creatinine 2.8 mg/dL, range 0.9 to 8.0), proteinuria in 100% (mean urine protein 5.8 g/day; range 1.9 to 13.0), nephrotic syndrome in 44%, and microhematuria in 60%. A monoclonal serum protein with the same heavy and light chain isotype as that of the glomerular deposits was identified in 50% of cases (including three IgGkappa and two IgGlambda); however, no patient had clinical or laboratory features of type 1 cryoglobulinemia. No patient had overt myeloma or lymphoma at presentation or over the course of follow-up (mean 12 months). Glomerular deposition of monoclonal IgG can produce a proliferative glomerulonephritis that mimics immune-complex glomerulonephritis by light and electron microscopy. Proper recognition of this entity requires confirmation of monoclonality by staining for the gamma heavy chain subclasses.
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            Paraneoplastic glomerulopathies: new insights into an old entity.

            P Ronco (1999)
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              Glomerulonephritis in chronic lymphocytic leukemia and related B-cell lymphomas.

              We retrospectively analyzed clinical presentation, immunopathological data and renal outcome in 13 patients with glomerulonephritis (GN) and chronic lymphocytic leukemia (CLL) or related diffuse well-differentiated lymphocytic lymphoma (WDLL) of B-cell lineage. B-cell proliferation and glomerulopathy were simultaneously diagnosed in seven of the 13 patients. Nephrotic syndrome was observed in nine patients. Serum creatinine was elevated (greater than 120 mumol/liter) in 10 patients and exceeded 400 mumol/liter in three patients. A clear cut relationship between GN and hematologic disease could be established in nine cases: five patients had MPGN caused by type I or type II cryoglobulinemia; two had MPGN or mesangial hypertrophy with circulating and deposited noncryoprecipitating monoclonal IgG K and IgM K, respectively; in the two remaining patients, monotypic IgG K glomerular deposits exhibiting fibrillary organization were observed in association with MGN or MPGN, despite the absence of circulating M-component by immunofixation. The pathophysiologic link between glomerular lesions and B-cell proliferation was further evidenced by effectiveness of specific treatment of the malignancy by chlorambucil. This drug, used in the absence of steroids, induced complete remission of nephrotic syndrome in the five patients to whom it was given. Moreover, in the five patients with creatininemia greater than 200 mumol/liter who received chemotherapy, substantial improvement in renal function was observed. These overall data demonstrate that the occurrence of GN in B-CLL and related lymphoma is not fortuitous, and testify to the paraneoplastic nature of glomerular involvement mediated by deposition and possibly processing of cryoprecipitating or noncryoprecipitating M-components.
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                Author and article information

                Journal
                Cases J
                Cases Journal
                BioMed Central
                1757-1626
                2009
                15 September 2009
                : 2
                : 7201
                Affiliations
                [1 ]University of Tennessee Health Science Center, Department of Internal Medicine, 956 Court Avenue, Room H314, Memphis, TN 38163, USA
                [2 ]Veterans Affairs Medical Center and Department of Medicine, 1030 Jefferson Avenue, Suite G 410, Memphis, TN 38103, USA
                Article
                1757-1626-0002-0000007201
                10.4076/1757-1626-2-7201
                2827065
                20181193
                68a98194-dfc1-4e64-86b7-ea681a0cd5c0
                Copyright ©2009 Alshayeb and Wall; licensee Cases Network Ltd. licensee BioMed Central Ltd.

                This is an Open Access article distributed under the terms of the Creative Commons Attribution License ( http://creativecommons.org/licenses/by/3.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                : 5 May 2009
                : 13 August 2009
                Categories
                Research article

                Medicine
                Medicine

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