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Light Chain Deposition Disease

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Glomerulonephritis

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Abstract

Monoclonal immunoglobulin deposition disease (MIDD) is a rare disorder characterized by the aberrant production and deposition of light and/or heavy chain immunoglobulins by clonal B-cell or plasma cell populations, resulting in multisystemic organ dysfunction. Renal disease is the most common initial disease manifestation, but other organs, such as the heart, lungs, liver, peripheral nerves, and skin, can be involved. Patients often present with nephrotic syndrome, hypertension, and an impaired glomerular filtration rate. Renal biopsy classically reveals nodular glomerulosclerosis on light microscopy, with non-congophilic, monotypic immunoglobulin deposits along the tubular and glomerular basement membranes by immunofluorescence and powdery and granular deposits on the basement membrane by electron microscopy. MIDD can be associated with multiple myeloma but may also be associated with a monoclonal gammopathy of renal significance, aparaprotein disorder of the kidney in patients who do not meet criteria for systemic lymphoma or multiple myeloma. The pathogenesis is related to an underlying clonal cell B or plasma cell population, the amount and primary structure of the abnormal light or heavy chain, as well as the interaction of the immunoglobulin with the local tissue environment. Treatment includes systemic chemotherapy and autologous stem cell transplantation to reduce the production of abnormal immunoglobulins. Bortezomib-based regimens have shown promise as a first-line therapy in MIDD. Renal transplantation has shown variable results due to the risk of recurrent disease.

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Authors and Affiliations

  1. Division of Nephrology and Hypertension, Mayo Clinic, Rochester, MN, USA

    Andrea Kattah & Nelson Leung

  2. Division of Hematology, Mayo Graduate School of Medicine, Mayo Clinic, 200 First St. SW, Rochester, MN, 55905, USA

    Nelson Leung

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  1. Andrea Kattah
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  2. Nelson Leung
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Correspondence to Nelson Leung .

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Editors and Affiliations

  1. Department of Pediatrics, NYU Langone Medical Center, New York, New York, USA

    Howard Trachtman

  2. Hospital of the Univ. of Pennsylvania, Philadelphia, Pennsylvania, USA

    Jonathan J. Hogan

  3. Cleveland Clinic, Cleveland, Ohio, USA

    Leal Herlitz

  4. Nephrology, University of Illinois at Chicago College of Medicine, Chicago, Illinois, USA

    Edgar V. Lerma

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Kattah, A., Leung, N. (2016). Light Chain Deposition Disease. In: Trachtman, H., Hogan, J., Herlitz, L., Lerma, E. (eds) Glomerulonephritis. Springer, Cham. https://doi.org/10.1007/978-3-319-27334-1_39-1

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