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Light Chain Deposition Disease: A Morphological Case Report

Light chain deposition disease (LCDD) is a rare condition associated with the overproduction and deposition of monoclonal light chain immunoglobulins. The kidneys are universally affected in LCDD, with the development of renal failure with nephrotic syndrome, microscopic hematuria, and proteinuria....

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Detalles Bibliográficos
Autores principales: Popov, Hristo, Stoyanov, George S, Ghenev, Peter
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Cureus 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9326408/
https://www.ncbi.nlm.nih.gov/pubmed/35903571
http://dx.doi.org/10.7759/cureus.26357
Descripción
Sumario:Light chain deposition disease (LCDD) is a rare condition associated with the overproduction and deposition of monoclonal light chain immunoglobulins. The kidneys are universally affected in LCDD, with the development of renal failure with nephrotic syndrome, microscopic hematuria, and proteinuria. Most cases are associated with a plasma cell neoplasm (multiple myeloma or plasmacytoma) or other lymphoproliferative disorders, with a reasonable number of cases also being idiopathic. Other organs can be affected in rare cases, without producing significant symptoms, predominantly the liver and heart. In this report, we discuss a case of a 72-year-old female presenting with the aforementioned symptoms. Percutaneous ultrasound-guided kidney biopsy revealed nodular sclerosis with periodic acid-Schiff stain (PAS)-positive, Congo red-negative, silver impregnation weakly-positive, and Masson's trichrome-positive (red reaction) deposits with ribbon-like changes together with light chain deposits in the tubular basement membranes. As LCDD was diagnosed, the patient was subjected to further tests, with multiple myeloma of the thoracic vertebrae also being diagnosed. The patient was started on myeloma treatment; however, she subsequently developed a severe lower limb infection that required amputation, after which she developed sepsis and expired.