Atypical renal involvement in Light Chain Amyloidosis
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Keywords

light chain amyloidosis
immunofluorescence
kidney disease
dialysis therapy

How to Cite

1.
Kuratomi Nakamura K, Mejía AF, Valderrama Cometa LA, Casanova Valderrama ME. Atypical renal involvement in Light Chain Amyloidosis. Rev. Colomb. Nefrol. [Internet]. 2022 Jun. 29 [cited 2022 Aug. 13];9(2). Available from: https://revistanefrologia.org/index.php/rcn/article/view/559

Abstract

Introduction: Light chain amyloidosis is an entity triggered by the proliferation of a clone of plasma cells that generates the accumulation of light chains, which are deposited in the form of amyloid fibrils generating organic dysfunction. Renal compromise generally manifests as nephrotic syndrome, with a slow and progressive decline of renal function that can lead to dialysis therapy.

Purpose: The objective of this case report is to demonstrate the aggressive and subacute renal involvement of systemic amyloidosis.

Case presentation: We present a case of a 35-year-old male patient who consulted for general malaise, blurred vision, dizziness and oliguria with elevated nitrogen levels that progressed fastly to the requirement of hemodialysis in approximately 3 months. It was also reveal peripheral polyneuropathy, cardiac infiltration, and slight elevation of transaminases. Given the negativity of extension studies, amyloid deposits were documented histopathologically with positive immunofluorescence for LA. He is currently receiving chemotherapy regimen with adequate clinical stability and tolerance.

Conclusion and discussion: To conclude, AL amyloidosis is a rare entity, with significant multi-organ involvement and high rates of morbidity and mortality. In this case, the subacute involvement with an early requirement for dialysis therapy is emphasized, and the importance of suspicion and timely diagnosis in patients with kidney involvement and other systemic manifestations is emphasized.

https://doi.org/10.22265/acnef.9.2.559
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References

Merlini G, Stone MJ. Dangerous small B-cell clones. Blood. 2006;108:2520-30. https://doi.org/10.1182/blood-2006-03-001164

Merlini G. AL amyloidosis: From molecular mechanisms to targeted therapies. Hematology. 2017 dic. 8;(1):1-12. https://doi.org/10.1182/asheducation-2017.1.1

Palladini G, Merlini G. What is new in diagnosis and management of light chain amyloidosis? Blood. 2016;128:159-68. https://doi.org/10.1182/blood-2016-01-629790

Pinney JH, Smith CJ, Taube JB, Lachmann HJ, Venner CP, Gibbs SDJ, et al. Systemic Amyloidosis in England: An epidemiological study. Br J Haematol. 2013 my.;161(4):525-32. https://doi.org/10.1111/bjh.12286

Wechalekar AD, Gillmore JD, Hawkins PN. Systemic amyloidosis. Lancet. 2016;387:2641-54. https://doi.org/10.1016/S0140-6736(15)01274-X

Tang W, McDonald SP, Hawley CM, Badve S V, Boudville N, Brown FG, et al. End-stage renal failure due to amyloidosis: Outcomes in 490 ANZDATA registry cases. Nephrol Dial Transplant. 2013 febr.;28(2):455-61. https://doi.org/10.1093/ndt/gfs492

Fine NM, Davis MK, Anderson K, Delgado DH, Giraldeau G, Kitchlu A, et al. Canadian Cardiovascular Society/Canadian Heart Failure Society Joint Position Statement on the Evaluation and Management of Patients With Cardiac Amyloidosis. Can J Cardiol. 2020 mzo. 1;36(3):322-34. https://doi.org/10.1016/j.cjca.2019.12.034

Kumar S, Dispenzieri A, Lacy M, Hayman S, Buadi F, Colby C, et al. Revised prognostic staging system for light chain amyloidosis incorporating cardiac biomarkers and serum free light chain measurements. J Clin Oncol. 2012 mzo. 20;30(9):989-95. https://doi.org/10.1200/JCO.2011.38.5724

Barrett CD, Dobos K, Liedtke M, Tuzovic M, Haddad F, Kobayashi Y, et al. A Changing Landscape of Mortality for Systemic Light Chain Amyloidosis. JACC Hear Fail. 2019 nov. 1;7(11):958-66. https://doi.org/10.1016/j.jchf.2019.07.007

Glenner GG. Amyloid Deposits and Amyloidosis. N Engl J Med. 1980 jun. 5;302(23):1283-92. https://doi.org/10.1056/NEJM198006053022305

Gertz MA, Lacy MQ, Dispenzieri A. Immunoglobulin light chain amyloidosis and the kidney. Kidney Int. 2002;61(1):1-9. https://doi.org/10.1046/j.1523-1755.2002.00085.x

Barbosa-de Carvalho NM, Morais-Sarmento ML. Free light chains assay in monoclonal gammopathies. Rev Hematol Mex. 2010;11(4):199-207.

Kyle RA, Greipp PR. Amyloidosis (AL). Clinical and laboratory features in 229 cases. Mayo Clin Proc. 1983 oct;58(10):665-83.

Kyle RA, Gertz MA. Primary systemic amyloidosis: clinical and laboratory features in 474 cases. Semin Hematol. 1995;32(1):45-59.

Vrana JA, Gamez JD, Madden BJ, Theis JD, Bergen HR, Dogan A. Classification of amyloidosis by laser microdissection and mass spectrometry-based proteomic analysis in clinical biopsy specimens. Blood. 2009 dic. 3;114(24):4957-9. https://doi.org/10.1182/blood-2009-07-230722

Palladini G, Dispenzieri A, Gertz M, Kumar S, Wechalekar A, Hawkins P, et al. New criteria for response to treatment in immunoglobulin light chain amyloidosis based on free light chain measurement and cardiac biomarkers: impact on survival outcomes. J Clin Oncol. 2012 dic. 20;30(36):4541-9. https://doi.org/10.1200/JCO.2011.37.7614

Tálamo G, Muhammad AM, Pandey MK, Zhu J, Creer MH, Malysz J. Estimation of Daily Proteinuria in Patients with Amyloidosis by Using the Protein-To-Creatinine ratio in Random Urine Samples. Rare Tumors. 2015 dic. 1;7(1):16-9. https://doi.org/10.4081/rt.2015.5686

Fogo AB, Lusco MA, Najafian B, Alpers CE. AJKD Atlas of Renal Pathology: AL Amyloidosis. Am J Kidney Dis. 2015;66:e43-5. https://doi.org/10.1053/j.ajkd.2015.10.006

Bergesio F, Ciciani AM, Santostefano M, Brugnano R, Manganaro M, Palladini G, et al. Renal involvement in systemic amyloidosis--an Italian retrospective study on epidemiological and clinical data at diagnosis. Nephrol Dial Transplant. 2007 jun.;22(6):1608-18. https://doi.org/10.1093/ndt/gfm041

Dispenzieri A. Clinical presentation, laboratory manifestations, and diagnosis of immunoglobulin light chain (AL) amyloidosis. UpToDate. Disponible en: https://www.uptodate.com/contents/clinical-presentation-laboratory-manifestations-and-diagnosis-of-immunoglobulin-light-chain-al-amyloidosis

Shi J, Guan J, Jiang B, Brenner DA, Del Monte F, Ward JE, et al. Amyloidogenic light chains induce cardiomyocyte contractile dysfunction and apoptosis via a non-canonical p38? MAPK pathway. Proc Natl Acad Sci. 2010 mzo. 2;107(9):4188-93. https://doi.org/10.1073/pnas.0912263107

Kyle RA, Linos A, Beard CM, Linke RP, Gertz MA, O'Fallon WM, et al. Incidence and natural history of primary systemic amyloidosis in Olmsted County, Minnesota, 1950 through 1989. Blood. 1992;79(7):1817-22.

Leukemia & Lymphoma Society. Trasplante de células madre sanguíneas y de médula ósea. Nueva York: LLS; 2015.

Madan S, Kumar SK, Dispenzieri A, Lacy MQ, Hayman SR, Buadi FK, et al. High-dose melphalan and peripheral blood stem cell transplantation for light-chain amyloidosis with cardiac involvement. Blood. 2012 febr. 2;119(5):1117-22. https://doi.org/10.1182/blood-2011-07-370031

Gertz MA. How to manage primary amyloidosis. Leukemia. 2012 febr.;26(2):191-8. https://doi.org/10.1038/leu.2011.219

Kastritis E, Leleu X, Arnulf B, Zamagni E, Cibeira MT, Kwok F, et al. Bortezomib, melphalan, and dexamethasone for light-chain amyloidosis. J Clin Oncol. 2020 oct. 1;38(28):3252-60. https://doi.org/10.1200/JCO.20.01285

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