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Monoclonal Immunoglobulin Deposition Disease

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Core Concepts in Parenchymal Kidney Disease

Abstract

Monoclonal immunoglobulin deposition disease (MIDD) is a systemic disorder characterized by the deposition of immunoglobulins as non-fibrillar material in various organ systems. Light-chain deposition disease (LCDD) was the first disease recognized in this spectrum, though several other entities, including heavy-chain deposition disease and light- and heavy-chain deposition disease, have since been described. MIDD often presents as renal dysfunction and nephrotic syndrome, and therefore the diagnosis is typically made by renal biopsy, though MIDD can affect the heart, lungs, liver, and central nervous system as well. In the majority of cases, renal biopsy demonstrates the deposition of kappa light chain by immunofluorescence, as opposed to light-chain amyloidosis where the lambda light chain predominates. The pathogenesis of MIDD is likely related to a combination of factors, including the properties of the light chain itself, the amount of light chain produced, and the reaction of the specific organ involved. The natural history and prognosis of MIDD depends on the severity of renal failure at presentation and the presence or absence of multiple myeloma. Several treatment options exist, including multidrug chemotherapeutic regimens, bortezomib (a proteasome inhibitor), and autologous stem-cell transplant. Renal transplantation in this population of patients remains controversial due to the high relapse rate, though more effective treatment regimens aimed at inducing complete remission prior to transplant may allow these patients to be transplanted successfully in the future.

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Acknowledgments

We would like to thank Dr. Lynn Cornell and Dr. Sanjeev Sethi from the Department of Pathology at the Mayo Clinic for providing the above figures.

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Authors and Affiliations

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

    Andrea G. Kattah M.D. & Nelson Leung M.D.

  2. Division of Hematology, Mayo Clinic, Rochester, MN, USA

    Nelson Leung M.D.

Authors
  1. Andrea G. Kattah M.D.
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  2. Nelson Leung M.D.
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Correspondence to Nelson Leung M.D. .

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Editors and Affiliations

  1. Div. Nephrology & Hypertension, Mayo Clinic Dept. Internal Medicine, Rochester, Minnesota, USA

    Fernando C. Fervenza

  2. Renal Division, Brigham and Women's Hospital, Boston, Massachusetts, USA

    Julie Lin

  3. Mayo Clinic, Rochester, Minnesota, USA

    Sanjeev Sethi

  4. Renal Division, Brigham and Women's Hospital Harvard Medical School, Boston, Massachusetts, USA

    Ajay K. Singh

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Kattah, A.G., Leung, N. (2014). Monoclonal Immunoglobulin Deposition Disease. In: Fervenza, F., Lin, J., Sethi, S., Singh, A. (eds) Core Concepts in Parenchymal Kidney Disease. Springer, New York, NY. https://doi.org/10.1007/978-1-4614-8166-9_20

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