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A Case of Light Chain Deposition Disease Leading to Acute Liver Failure and Review of Literature
Light chain deposition disease (LCDD) is a monoclonal immunoglobulin deposition disease characterized by light chain deposition in soft tissues and viscera, causing systemic organ dysfunction with an underlying lymphoproliferative disorder. While the kidney is the most affected organ, cardiac and he...
Autores principales: | , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
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MDPI
2023
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9944111/ https://www.ncbi.nlm.nih.gov/pubmed/36810539 http://dx.doi.org/10.3390/diseases11010024 |
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author | Gandhi, Mustafa Pasha, Syed Bilal Reznicek, Emily Pasha, Syed Raheel Ertugrul, Hamza Araslanova, Adel Yin, Feng Tahan, Veysel |
author_facet | Gandhi, Mustafa Pasha, Syed Bilal Reznicek, Emily Pasha, Syed Raheel Ertugrul, Hamza Araslanova, Adel Yin, Feng Tahan, Veysel |
author_sort | Gandhi, Mustafa |
collection | PubMed |
description | Light chain deposition disease (LCDD) is a monoclonal immunoglobulin deposition disease characterized by light chain deposition in soft tissues and viscera, causing systemic organ dysfunction with an underlying lymphoproliferative disorder. While the kidney is the most affected organ, cardiac and hepatic involvement is also seen with LCDD. Hepatic manifestation can range from mild hepatic injury to fulminant liver failure. Herein, we are presenting a case of an 83-year-old woman with a monoclonal gammopathy of undetermined significance (MGUS), who presented to our institution with acute liver failure progressing to circulatory shock and multiorgan failure. After an extensive workup, a diagnosis of hepatic LCDD was determined. In conjunction with the hematology and oncology department, chemotherapy options were discussed, but given her poor prognosis, the family decided to pursue a palliative route. Though establishing a prompt diagnosis is important for any acute condition, the rarity of this condition, along with paucity of data, makes timely diagnosis and treatment challenging. The available literature shows variable rates of success with chemotherapy for systemic LCDD. Despite chemotherapeutic advances, liver failure in LCDD indicates a dismal prognosis, where further clinical trials are difficult owing to the low prevalence of the condition. In our article, we will also be reviewing previous case reports on this disease. |
format | Online Article Text |
id | pubmed-9944111 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2023 |
publisher | MDPI |
record_format | MEDLINE/PubMed |
spelling | pubmed-99441112023-02-23 A Case of Light Chain Deposition Disease Leading to Acute Liver Failure and Review of Literature Gandhi, Mustafa Pasha, Syed Bilal Reznicek, Emily Pasha, Syed Raheel Ertugrul, Hamza Araslanova, Adel Yin, Feng Tahan, Veysel Diseases Case Report Light chain deposition disease (LCDD) is a monoclonal immunoglobulin deposition disease characterized by light chain deposition in soft tissues and viscera, causing systemic organ dysfunction with an underlying lymphoproliferative disorder. While the kidney is the most affected organ, cardiac and hepatic involvement is also seen with LCDD. Hepatic manifestation can range from mild hepatic injury to fulminant liver failure. Herein, we are presenting a case of an 83-year-old woman with a monoclonal gammopathy of undetermined significance (MGUS), who presented to our institution with acute liver failure progressing to circulatory shock and multiorgan failure. After an extensive workup, a diagnosis of hepatic LCDD was determined. In conjunction with the hematology and oncology department, chemotherapy options were discussed, but given her poor prognosis, the family decided to pursue a palliative route. Though establishing a prompt diagnosis is important for any acute condition, the rarity of this condition, along with paucity of data, makes timely diagnosis and treatment challenging. The available literature shows variable rates of success with chemotherapy for systemic LCDD. Despite chemotherapeutic advances, liver failure in LCDD indicates a dismal prognosis, where further clinical trials are difficult owing to the low prevalence of the condition. In our article, we will also be reviewing previous case reports on this disease. MDPI 2023-02-01 /pmc/articles/PMC9944111/ /pubmed/36810539 http://dx.doi.org/10.3390/diseases11010024 Text en © 2023 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/). |
spellingShingle | Case Report Gandhi, Mustafa Pasha, Syed Bilal Reznicek, Emily Pasha, Syed Raheel Ertugrul, Hamza Araslanova, Adel Yin, Feng Tahan, Veysel A Case of Light Chain Deposition Disease Leading to Acute Liver Failure and Review of Literature |
title | A Case of Light Chain Deposition Disease Leading to Acute Liver Failure and Review of Literature |
title_full | A Case of Light Chain Deposition Disease Leading to Acute Liver Failure and Review of Literature |
title_fullStr | A Case of Light Chain Deposition Disease Leading to Acute Liver Failure and Review of Literature |
title_full_unstemmed | A Case of Light Chain Deposition Disease Leading to Acute Liver Failure and Review of Literature |
title_short | A Case of Light Chain Deposition Disease Leading to Acute Liver Failure and Review of Literature |
title_sort | case of light chain deposition disease leading to acute liver failure and review of literature |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9944111/ https://www.ncbi.nlm.nih.gov/pubmed/36810539 http://dx.doi.org/10.3390/diseases11010024 |
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