Portal hypertension related to light chain deposition disease of liver: an enlightening experience

Talukdar, Arunansu; Mukherjee, Kasturi; Khanra, Dibbendhu; Saha, Manideepa

doi:10.1136/bcr-2013-009553

Cited by 2 publications

(1 citation statement)

References 20 publications

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“…After the heart, the liver is the most affected extrarenal organ [ 2 ], associated with varying degrees of hepatic dysfunction [ 8 , 9 , 10 , 11 , 12 , 13 , 14 , 15 , 16 , 17 ] There is usually concomitant renal involvement in those with severe liver disease. In those with established liver failure, the prognosis is guarded [ 10 , 11 ].…”

Section: Discussionmentioning

confidence: 99%

A Case of Light Chain Deposition Disease Leading to Acute Liver Failure and Review of Literature

et al. 2023

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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.

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