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Pathologies of Renal and Systemic Intracellular Paraprotein Storage: Crystalopathies and Beyond

  • Chapter
Amyloid and Related Disorders

Part of the book series: Current Clinical Pathology ((CCPATH))

Abstract

This chapter provides a brief overview of pathologies associated with intracellular paraproteins, both renal and systemic. The expanding spectrum of intracellular immunoglobulin storage pathologies, including both organized deposits (crystalline, rarely fibrillar) and non-organized deposits, is considered. In the kidney, earlier observations reported an association between Fanconi syndrome and the presence of prominent, kappa light chain restricted crystals in proximal tubular epithelium. However, more recent observations have included also noncrystalline light chain proximal tubulopathy, which may be more prevalent than the crystalline type and tends to be associated predominantly with lambda light chain restriction. To encompass this expanding spectrum of proximal tubular pathology, the term light chain proximal tubulopathy was proposed. In crystal-storing histiocytosis, the histiocytes contain prominent intralysosomal crystalline cytoplasmic immunoglobulin inclusions. However, more recently, noncrystallized forms of immunoglobulin-storing histiocytosis have also been recognized and the term immunoglobulin-storing histiocytosis has been proposed to encompass the expanding pathology. In certain plasma cell dyscrasias/B-cell lymphoproliferative disorders, similar crystalline and noncrystalline inclusions can also be seen in the neoplastic plasma/B-cells. Although the lesions discussed in this chapter are relatively rare, awareness of these lesions is important since they represent a useful indicator that can facilitate the early diagnosis of an underlying plasma cell dyscrasia/B-cell lymphoproliferative process and avoid misdiagnosis. While, in many instances, the clinical course may be indolent, significant target organ damage occurs over time. Although there is no consensus with regard to the best treatment options, elimination of the involved clone is associated with a reversal of target organ injury. Therefore, efforts to design effective treatments for these “small but dangerous” clones should continue.

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Authors and Affiliations

  1. Department of Pathology, Loyola University Medical Center, Loyola University Chicago, Building 110, Room 2242, 2160 South First Avenue, Maywood, IL, 60153, USA

    Maria M. Picken MD, PhD

  2. Departments of Pathology and Laboratory Medicine, Memorial Sloan Kettering Cancer Center, 1275 York Avenue, New York, NY, 10065, USA

    Ahmet Dogan MD, PhD

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Correspondence to Maria M. Picken MD, PhD .

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  1. Department of Pathology, Loyola University Medical Center, Maywood, Illinois, USA

    Maria M. Picken

  2. Department of Pathology, LSU Health Shreveport, Shreveport, Louisiana, USA

    Guillermo A. Herrera

  3. Department of Pathology and Laboratory Medicine, Department of Pathology and Laboratory Medicine, New York, New York, USA

    Ahmet Dogan

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Picken, M.M., Dogan, A. (2015). Pathologies of Renal and Systemic Intracellular Paraprotein Storage: Crystalopathies and Beyond. In: Picken, M., Herrera, G., Dogan, A. (eds) Amyloid and Related Disorders. Current Clinical Pathology. Humana Press, Cham. https://doi.org/10.1007/978-3-319-19294-9_12

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