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Light-Chain Deposition Disease: Diagnosis, Prognosis and Therapy

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Abstract

Light-chain deposition disease (LCDD) is a systemic disease characterized by monotypic light-chain (LC) deposits. Although various organs may be affected, it is usually heralded by renal involvement. Diagnosis is made on immunohistologic findings: deposits are reactive with one anti-light-chain antiserum (kappa or lambda) and lack amyloid characteristics. LCDD is always associated with B-cell dyscrasias, but in one third of reported cases the lymphoplasmacytic proliferation does not fit the usual criteria for malignancy. In this article we review the prominent pathological and clinical features that are currently important to consider in the clinical care of patients with LCDD.

Keywords

  • Light Chain
  • Deposition Disease
  • Plasma Cell Leukemia
  • Peliosis Hepatis
  • Light Chain Deposition Disease

These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

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References

  1. Ivanyi B: Frequency of light chain deposition nephropathy relative to renal amyloidosis and Bence-Jones cast nephropathy in a necropsy study of patients with myeloma. Arch Pathol Lab Med, 114: 986–987, 1990.

    PubMed  CAS  Google Scholar 

  2. Fang LST: Light-chain nephropathy. Kidney Int, 27: 582–592, 1985.

    PubMed  CrossRef  CAS  Google Scholar 

  3. Kyle RA: Monoclonal gammopathies and the kidney. Ann Rev Med, 40: 53–60, 1989.

    PubMed  CrossRef  CAS  Google Scholar 

  4. Sturgill BC, Tucker FL, Bolton WK: Immunoglobulin light chain nephropathies. Pathol Annu, 22: 133–150, 1987.

    PubMed  Google Scholar 

  5. Hill GS: Dysproteinemias, amyloidosis and immunotactoid glomerulopathy. In: “Pathology of the kidney” (Ed RH Hepstinstall), Vol. 2, Little Brown, Boston, 1991, pp 1631–1713.

    Google Scholar 

  6. Noël LH, Droz D, Ganeval D, Grünfeld JP: Renal granular monoclonal light chain deposits: morphological aspects in 11 cases. Gin Nephrol, 21: 263–269, 1984.

    Google Scholar 

  7. Solomon A, Weiss DT, Kattine AA: Nephrotoxic potential of Bence-Jones proteins. N Engl J Med, 324: 1845–1851, 1991.

    PubMed  CrossRef  CAS  Google Scholar 

  8. Sanders PW, Herrera GA, Kirk KA, Old CW, Galla JH: Spectrum of glomerular and tubulointerstitial renal lesions associated with monoclonal immunoglobulin light chain deposition. Lab Invest, 64: 527–537, 1991.

    PubMed  CAS  Google Scholar 

  9. Ganeval D, Noel LH, Preud’phomme JL, Droz D, Grünfeld JP: Light-chain deposition disease: its relation with Al-type amyloidosis. Kidney Int, 26:1–9, 1984.

    PubMed  CrossRef  CAS  Google Scholar 

  10. Sinniah R, Cohen AM: Glomerular capillary aneurysms in light-chain nephropathy. Am J Pathol, 118:298–305, 1985.

    PubMed  CAS  Google Scholar 

  11. Bangertner AR, Murphy WM: Kappa light chain nephropathy. Virchows Arch A, 410: 531–539, 1987.

    Google Scholar 

  12. Alpers CE: Immunotactoid glomerulopathy: an entity distinct from fibrillary glomerulonephritis. Am J Kidney Dis, 19: 185–191, 1992.

    PubMed  CAS  Google Scholar 

  13. Gallo G, Picken M, Buxbaum J, Frangione B: The spectrum of monoclonal immunoglobulin deposition disease associated with immunocytic dyscrasias. Semin Hematol, 26: 234–245, 1989.

    PubMed  CAS  Google Scholar 

  14. Randall RE, Williamson WC, Mullinax F, Tung MY, Still WJS: Manifestations of systemic light chain deposition. Am J Med, 60: 293–299, 1980.

    CrossRef  Google Scholar 

  15. Buxbaum JN, Chuba JV, Hellman GC, Solomon AL, Gallo GR: Monoclonal immunoglobulin deposition disease: light chain and light and heavy chain deposition diseases and their relation to light chain amyloidosis. Ann Int Med, 112: 455–464, 1990.

    PubMed  CAS  Google Scholar 

  16. Droz D, Noel LH, Carnot F, Degos F, Ganeval D, Grünfeld JP: Liver involvement in nonamyloid light chain deposits disease. Lab Invest, 50: 683–689, 1984.

    PubMed  CAS  Google Scholar 

  17. Voinchet O, Degott C, Scoazec JY, Feldmann G, Benhamou JP: Peliosis hepatis, nodular regenerative hyperplasia of the liver and light-chain deposition in a patient with Waldenström’s macroglobulinemia. Gastroenterology, 95: 482–486, 1988.

    PubMed  CAS  Google Scholar 

  18. Dalakas MC, Engel WK: Polyneuropathy with monoclonal gammopathies: studies of 11 patients. Ann Neurol, 10: 45–52, 1981.

    PubMed  CrossRef  CAS  Google Scholar 

  19. Kijner CM, Yousem SA: Systemic light chain deposition disease presenting as multiple pulmonary nodules. Am J Surg Pathol, 12: 405–413, 1988.

    PubMed  CrossRef  CAS  Google Scholar 

  20. Tubbs RR, Gephardt GN, McNahon JT, Hall PM, Valenzuela R, Vidt DG: Light chain nephropathy. Am J Med, 71: 263–269, 1981.

    PubMed  CrossRef  CAS  Google Scholar 

  21. Confalioneri R, Barbiano di Belgiojoso G, Banfi G, Ferrario F, Bertani T, Pozzi C, Casanova S, Lupo A, de Ferrari G, Minetti L: Light chain nephropathy: histological and clinical aspects in 15 cases. Nephrol Dial Transplant, 2: 150–156, 1988.

    Google Scholar 

  22. Nakamato Y, Imai H, Hamanaka S, Yoshida K, Akihama T, Miura AB: IgM monoclonal gammopathy accompanied by nodular glomerulosclerosis, urine-concentrating defect and hyporeninemic hypoaldosteronism. Am J Nephrol, 5: 53–58, 1985.

    CrossRef  Google Scholar 

  23. Peng SK, French WJ, Cohen AH, Fausel RE: Light chain cardiomyopathy associated with small-vessel disease. Arch Pathol Lab Med, 112: 844–846, 1988.

    PubMed  CAS  Google Scholar 

  24. Bedossa P, Fabre M, Paraf F, Martin E, Lemaigre G: Light chain deposition disease with liver dysfunction. Human Pathol, 19: 1008–1014, 1988.

    CrossRef  CAS  Google Scholar 

  25. Faa G, Van Eyken P, De Vos R, Fevery J, Van Damme B, De Groote J, Desmet VJ: Light chain deposition disease of the liver associated with AL-type amyloidosis and severe cholestasis. J Hepatol, 12: 75–82, 1991.

    PubMed  CrossRef  CAS  Google Scholar 

  26. Maury CPJ, Teppo AM: Massive cutaneous hyalinosis. Am J Clin Pathol, 82: 543–551, 1984.

    PubMed  CAS  Google Scholar 

  27. Rahman A, Mossey RT, Susin M, Budman D, Mailloux LU: Kappa-chain nephropathy associated with plasma cell leukemia. Arch Int Med, 144: 1689–1690, 1984.

    CrossRef  CAS  Google Scholar 

  28. Preud’homme JL: Immunoglobulin synthesis in plasma cell dyscrasias with renal lesions. In: “The kidney in plasma cell dyscrasias” (Eds L Minetti, G D’Amico, C Ponticelli), Kluwer Academic Publishers, Dordrecht, The Netherlands, 1988, pp 31–43.

    CrossRef  Google Scholar 

  29. Matsuzaki H, Yoshida M, Akahoshi Y, Kuwahara K, Satout T, Takatsuki K: Pseudo-nonsecretory multiple myeloma with light chain deposition disease. Acta Haematol, 85: 164–168, 1991.

    PubMed  CrossRef  CAS  Google Scholar 

  30. Heilman RL, Holley K, Offord K, Velosa J, Kyle R: Apparent renal response to melphalan and prednisone (MP) in light chain deposition disease. Kidney Int, 35: 209A, 1988 (Abstract).

    Google Scholar 

  31. Gipstein RM, Cohen AH, Adams DA, Adams T, Grabie MT: Kappa light chain nephropathy without evidence of myeloma cells. Response to chemotherapy with cessation of maintenance hemodialysis. Am J Nephrol, 2: 276–281, 1982.

    PubMed  CrossRef  CAS  Google Scholar 

  32. Alpers CE, Marchioro TL, Johnson RJ: Monoclonal immunoglobulin deposition disease in a renal allograft: probable recurrent disease in a patient without myeloma. Am J Kidney Dis, 13: 418–423, 1989.

    PubMed  CAS  Google Scholar 

  33. Gerlag PGG, Koene RAP, Berden JHM: Renal transplantation in light chain nephropathy: case report and review of the literature. Clin Nephrol, 25: 101–104, 1986.

    PubMed  CAS  Google Scholar 

  34. Picken MM, Frangione B, Barlogie B, Luna M, Gallo G: Light chain deposition disease derived from the K1 light chain subgroup. Am J Pathol, 134: 749–754, 1989.

    PubMed  CAS  Google Scholar 

  35. Cogne M, Preud’homme JL, Bauwens M, Touchard G, Aucouturier P: Structure of a monoclonal kappa chain of the VKIV subgroup in the kidney and plasma cells in light chain deposition disease. J Clin Invest, 87: 2188–2190, 1991.

    CrossRef  Google Scholar 

  36. Khamlichi AA, Aucouturier P, Silvain C, Bauwens M, Touchard G, Preud’homme JL, Nau F, Cogne M: Primary structure of a monoclonal k chain in myeloma with light chain deposition disease. Clin Exp Immunol, 87: 122–126, 1992.

    PubMed  CrossRef  CAS  Google Scholar 

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© 1992 Springer-Verlag London Limited

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Chauveau, D., Ganeval, D. (1992). Light-Chain Deposition Disease: Diagnosis, Prognosis and Therapy. In: Andreucci, V.E., Fine, L.G. (eds) International Yearbook of Nephrology 1993. Springer, London. https://doi.org/10.1007/978-1-4471-1948-7_3

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  • DOI: https://doi.org/10.1007/978-1-4471-1948-7_3

  • Publisher Name: Springer, London

  • Print ISBN: 978-1-4471-1950-0

  • Online ISBN: 978-1-4471-1948-7

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