Intrahepatic cholestasis as the initial manifestation of systemic light-chain amyloidosis (AL)
DOI:
https://doi.org/10.32818/reccmi.a10n3a16Keywords:
light chain amyloidosis, hepatic amyloidosis, cholestasis, daratumumab, storage diseases, liver diseasesAbstract
Light-chain (AL) amyloidosis is a rare systemic disorder characterized by the extracellular deposit of misfolded monoclonal immunoglobulins, which can affect multiple organs. We report an unusual case of AL amyloidosis presenting with initial hepatic involvement, diagnosed through liver biopsy that confirmed κ-type AL amyloidosis. Early recognition allowed initiation of tailored therapy with daratumumab and dexamethasone, resulting in a favorable clinical outcome. This case underscores the significance of timely diagnosis in hepatic amyloidosis, enabling improved prognosis and optimized therapeutic options.
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Kumar S, Dispenzieri A, Katzmann JA, Larson DR, Colby CL, Lacy MQ, et al. Serum immunoglobulin free light-chain measurement in primary amyloidosis: prognostic value and correlations with clinical features. Blood. 2010; 116(24): 5126–29. doi: https://doi.org/10.1182/blood-2010-06-290668 (último acceso dic. 2025). DOI: https://doi.org/10.1182/blood-2010-06-290668
Park MA, Mueller PS, Kyle RA, Larson DR, Plevak MF, Gertz MA. Primary (AL) hepatic amyloidosis: clinical features and natural history in 98 patients. Medicine (Baltimore). 2003; 82(5): 291–298. doi: https://doi.org/10.1097/01.md.0000091183.93122.c7 (último acceso dic. 2025). DOI: https://doi.org/10.1097/01.md.0000091183.93122.c7
Nakano Y, Kawamoto R, Ito E, Matukawa K. A case of cholestatic liver involvement secondary to amyloid light chain amyloidosis with new-onset hypercholesterolemia and elevated gamma-glutamyltransferase level. Cureus. 2023; 15(8): e44001. doi: https://doi.org/10.7759/cureus.44001 (último acceso dic. 2025). DOI: https://doi.org/10.7759/cureus.44001
Tarakji R, Martin P, Perreault G. Amyloidosis and the liver. Clin Liver Dis. 2025; 29(3): 371-84. doi: https://doi.org/10.1111/jgh.70083 (último acceso dic. 2025). DOI: https://doi.org/10.1016/j.cld.2025.03.007
Yasir S, Chen ZE, Hartley C, Zhang L, Torbenson M. Morphological findings in different subtypes of hepatic amyloid. Hum Pathol. 2024; 146: 35-42. doi: https://doi.org/10.1016/j.humpath.2024.03.001 (último acceso dic. 2025). DOI: https://doi.org/10.1016/j.humpath.2024.03.001
Kyle RA, Gertz MA. Primary systemic amyloidosis: clinical and laboratory features in 474 cases. Semin Hematol. 1995; 32(1): 45–59. Accesible en: http://www.smoczynski.com/strona_Bartka/am_pl/Publications/a.pdf (último acceso dic. 2025).
Peters RA, Koukoulis G, Gimson AE, Portmann B, Westaby D, Williams R. Primary amyloidosis and severe intrahepatic cholestatic jaundice. Gut. 1994; 35(9): 1322–25. doi: https://doi.org/10.1136/gut.35.9.1322 (último acceso dic. 2025). DOI: https://doi.org/10.1136/gut.35.9.1322
Cardoso BA, Alves R, Leal R, Caetano A, Ferreira T, Campilho F, et al. Primary hepatic amyloidosis presenting as acute liver failure. BMJ Case Rep. 2016; 2016: bcr2016214392. doi: https://doi.org/10.1136/bcr-2016-214392 (último acceso dic. 2025). DOI: https://doi.org/10.1136/bcr-2016-214392
Kastritis E, Palladini G, Minnema MC, Wechalekar AD, Jaccard A, Lee HC, et al. Daratumumab-based treatment for immunoglobulin light-chain amyloidosis. N Engl J Med. 2021; 385(1): 46–58. doi: https://doi.org/10.1056/NEJMoa2028631 (último acceso dic. 2025). DOI: https://doi.org/10.1056/NEJMoa2028631
Kittleson MM, Ruberg FL, Ambardekar AV, Brannagan TH, Cheng RK, Clarke JO, et al. 2023 ACC expert consensus decision pathway on comprehensive multidisciplinary care for the patient with cardiac amyloidosis: a report of the American College of Cardiology Solution Set Oversight Committee. J Am Coll Cardiol. 2023; 81(11): 1076–26. doi: https://doi.org/10.1016/j.jacc.2022.11.022 (último acceso dic. 2025). DOI: https://doi.org/10.1016/j.jacc.2022.11.022
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Copyright (c) 2025 Miriam Bragado Pascual, Rosanna Villanueva Hernández, Beatriz Segovia Blázquez, María Pilar Delgado Álvarez, Juan Manuel Blanco Esteban
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