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Immunoglobulin Deposition Diseases

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Biology and Management of Unusual Plasma Cell Dyscrasias

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Abstract

Immunoglobulin deposition diseases are rare and unusual plasma cell dyscrasias. A tissue biopsy is required for diagnosis. Since the kidney is involved in most cases, a kidney biopsy usually provides the diagnosis. It is important to distinguish these disorders as their clinical presentation often mimics multiple myeloma and amyloidosis. The degree of renal involvement at presentation may vary from mild renal impairment to severe renal dysfunction requiring renal replacement therapy. The underlying lymphoproliferative or clonal plasma cell disorder usually has a low tumor burden compared to conditions such as multiple myeloma or Waldenstrom’s macroglobulinemia. The identification of the underlying hematological disorder guides therapeutic intervention. There has been significant improvement in the outcome of these patients, since the availability of novel agents such as immunomodulatory drugs, proteasome inhibitors, and with autologous stem cell transplantation. In this review, we address clinical presentation and outcomes with light-chain and heavy-chain monoclonal immunoglobulin deposition disease, Type I cryoglobulinemia, proliferative glomerulonephritis with monoclonal IgG deposits, C3 monoclonal-associated glomerulonephritis, immunotactoid glomerulonephropathy, Fanconi syndrome, and light-chain crystallopathy.

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Acknowledgments

We thank Dr. Samih H. Nasr for providing the images used in this manuscript.

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Authors and Affiliations

  1. Division of Hematology, Department of Medicine, Mayo Clinic, 200 First St. SW, Rochester, MN, 55905, USA

    Vinay Gupta MD, Wilson I. Gonsalves MD & Francis K. Buadi MB, ChB

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  1. Vinay Gupta MD
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  2. Wilson I. Gonsalves MD
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  3. Francis K. Buadi MB, ChB
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Correspondence to Francis K. Buadi MB, ChB .

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Editors and Affiliations

  1. Section of Hematology/Oncology MC2115, University of Chicago, Chicago, USA

    Todd M. Zimmerman

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

    Shaji K. Kumar

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Gupta, V., Gonsalves, W.I., Buadi, F.K. (2017). Immunoglobulin Deposition Diseases. In: Zimmerman, T., Kumar, S. (eds) Biology and Management of Unusual Plasma Cell Dyscrasias. Springer, New York, NY. https://doi.org/10.1007/978-1-4419-6848-7_6

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  • DOI: https://doi.org/10.1007/978-1-4419-6848-7_6

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