Cargando…

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...

Descripción completa

Detalles Bibliográficos
Autores principales: Gandhi, Mustafa, Pasha, Syed Bilal, Reznicek, Emily, Pasha, Syed Raheel, Ertugrul, Hamza, Araslanova, Adel, Yin, Feng, Tahan, Veysel
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2023
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
_version_ 1784891845662212096
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
work_keys_str_mv AT gandhimustafa acaseoflightchaindepositiondiseaseleadingtoacuteliverfailureandreviewofliterature
AT pashasyedbilal acaseoflightchaindepositiondiseaseleadingtoacuteliverfailureandreviewofliterature
AT reznicekemily acaseoflightchaindepositiondiseaseleadingtoacuteliverfailureandreviewofliterature
AT pashasyedraheel acaseoflightchaindepositiondiseaseleadingtoacuteliverfailureandreviewofliterature
AT ertugrulhamza acaseoflightchaindepositiondiseaseleadingtoacuteliverfailureandreviewofliterature
AT araslanovaadel acaseoflightchaindepositiondiseaseleadingtoacuteliverfailureandreviewofliterature
AT yinfeng acaseoflightchaindepositiondiseaseleadingtoacuteliverfailureandreviewofliterature
AT tahanveysel acaseoflightchaindepositiondiseaseleadingtoacuteliverfailureandreviewofliterature
AT gandhimustafa caseoflightchaindepositiondiseaseleadingtoacuteliverfailureandreviewofliterature
AT pashasyedbilal caseoflightchaindepositiondiseaseleadingtoacuteliverfailureandreviewofliterature
AT reznicekemily caseoflightchaindepositiondiseaseleadingtoacuteliverfailureandreviewofliterature
AT pashasyedraheel caseoflightchaindepositiondiseaseleadingtoacuteliverfailureandreviewofliterature
AT ertugrulhamza caseoflightchaindepositiondiseaseleadingtoacuteliverfailureandreviewofliterature
AT araslanovaadel caseoflightchaindepositiondiseaseleadingtoacuteliverfailureandreviewofliterature
AT yinfeng caseoflightchaindepositiondiseaseleadingtoacuteliverfailureandreviewofliterature
AT tahanveysel caseoflightchaindepositiondiseaseleadingtoacuteliverfailureandreviewofliterature