Nonamyloidotic glomerular disease caused by light chain deposits: a case report
Keywords:
Kidney diseases, paraproteinemias, immunoglobulin light chains, case reports.
Abstract
The nephropathy of monoclonal gammopathies is principally caused by light chain deposits of fragmented immunoglobins. Paraprotein-related renal diseases are associated with such deposits of intact (heavy chain) or fragmentary (light chain) immunoglobins. A condition of pathological light chain deposits is rare and characterized by deposits of fragments of monoclonal immunoglobulins in many organs. Renal deposits occur primarily in glomeruli and tubular basement membranes. This disease is frequently associated with lymphoproliferative disorders. The majority of cases are caused by deposits of kappa light chains. Whereas this disease is most frequently associated with hematologic malignancies, occasionally a case occurs without detectable hematological pathologies; these cases are called idiopathic or primary. They usually manifest themselves as severe renal insufficiencies with nephrotic-range proteinuria. No treatment regime has been clearly established and the prognosis is poor. Herein, the clinical and histological characteristics are described regarding the first case in Colombia of light chain deposit disease without symptoms of malignancy.Downloads
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References
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11. Sanders PW. Management of paraproteinemic renal disease. Curr Opin Nephrol Hypertens. 2005;14:97-103.
12. García A, Aneiros J, Ramos AM, Petkov V, López JL, Albalate M, et al. Insuficiencia renal por enfermedad de depósito de cadenas ligeras. Nefrología. 2008;2:212-5.
13. Campistol JM. Amiloidosis renal y glomerulonefritis fibrilares. En: Avendaño LH, Aljama M, Arias M, Caramelo C, Egido JE, Lamas S, editores. Nefrología clínica. Madrid: Editorial Médica Panamericana S.A.; 2003. p. 387-93.
2. Ronco P, Plaisier E, Mougenot B, Aucouturier P. Immunoglobulin light (Heavy)-chain deposition disease: from molecular medicine to pathophysiology-driven therapy. Clin J Am Soc Nephrol. 2006;1:1342-50.
3. Ronco PM, Alyanakian MA, Mougenot B, Aucouturier P. Light chain deposition disease: A model of glomerulosclerosis defined at the molecular level. J Am Soc Nephrol. 2001;12:1558-65.
4. Antonovych TT, Lin RC, Parrish E. Light chain deposits in multiple myeloma. Lab Invest. 1974;30:370A.
5. Randall RE, Williamson WC Jr, Mullinax F, Tung MY, Still WJ. Manifestations of systemic light chain deposition. Am J Med. 1976;60:293-9.
6. Ronco P, Aucouturier P, Mougenot B. Kidney involvement in plasma cell dyscrasias. In: Davinson AM, editor. Oxford textbook of clinical nephrology. Third edition. New York, NY: Oxford University Press; 2005.
7. Martín C, Suñer M, Cabrera R, Díaz M, Fernández J. Enfermedad por depósito de cadenas ligeras. Expe-riencia en nuestro medio. Nefrología. 2008;5:539-42.
8. Ramos R, Poveda R, Bernís C, Ara J, Sunyer M, Arrizabalaga P, et al. Afectación renal en las gammapatías monoclonales benignas: ¿una entidad infradiagnosticada? Nefrología. 2008;5:525-30.
9. Santostefano M, Zanchelli F, Zaccaria A, Poletti G, Fusaroli M. The ultrastructural basis of renal pathology in monoclonal gammopathies. J Nephrol. 2005;18:659-75.
10. Ramos R, Poveda R, Sarrá J, Domingo A, Carreras L, Grinyó JM. Renal involvement in non-malignant IgM gammopathy. Nephrol Dial Transplant. 2007;22;627-30.
11. Sanders PW. Management of paraproteinemic renal disease. Curr Opin Nephrol Hypertens. 2005;14:97-103.
12. García A, Aneiros J, Ramos AM, Petkov V, López JL, Albalate M, et al. Insuficiencia renal por enfermedad de depósito de cadenas ligeras. Nefrología. 2008;2:212-5.
13. Campistol JM. Amiloidosis renal y glomerulonefritis fibrilares. En: Avendaño LH, Aljama M, Arias M, Caramelo C, Egido JE, Lamas S, editores. Nefrología clínica. Madrid: Editorial Médica Panamericana S.A.; 2003. p. 387-93.
How to Cite
1.
Cantillo J de J, López R del P, Andrade RE. Nonamyloidotic glomerular disease caused by light chain deposits: a case report. Biomed. [Internet]. 2009 Dec. 1 [cited 2025 Apr. 4];29(4):531-8. Available from: https://revistabiomedicaorg.biteca.online/index.php/biomedica/article/view/127
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Published
2009-12-01
Issue
Section
Case presentation
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