Light Chain Deposition Disease Presenting as Cholestatic Jaundice: A Case Report

Kumar, Prashanth Ashok; Sandhya, .; Venkatakrishnan, L; Krishnaveni, J; Mohanakrishnan, A; Nirmala,

doi:10.5001/omj.2012.12

Cited by 8 publications

(11 citation statements)

References 8 publications

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“…As with our case, LCs in hepatic LCDD are deposited in a perisinusoidal pattern that contrasts with amyloidosis, wherein a mostly parenchymal pattern of deposition is seen [ 13 ]. Immunofluorescence (IF) microscopy can often detect immunoglobulin light chains and C1q and C3 complement proteins [ 17 ] Electron microscopy demonstrates punctate granular deposits that can be diagnostic.…”

Section: Discussionmentioning

confidence: 52%

“…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%

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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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Section: Discussionmentioning

confidence: 52%

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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“…The deposits are non-amyloid, do not stain with Congo red and do not exhibit a fibrillar ultrastructure [1] . Hepatic LC deposits assume a perisinusoidal distribution, as opposed to amyloid deposits, mostly in liver parenchyma [2, 3] .…”

Section: Discussionmentioning

confidence: 99%

“…In our case, although there was also renal and heart involvement, the clinical picture was dominated by hepatic manifestations, which led to a wider variety of differential diagnoses. This degree of hepatomegaly, severity of cholestasis and signs of portal hypertension are not very common in LCDD [3] .…”

Section: Discussionmentioning

confidence: 99%

Light-Chain Deposition Disease with Prominent Hepatic Involvement

Cristino

Pais

Silva

et al. 2017

European Journal of Case Reports in Internal Medicine

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Light-chain deposition disease (LCDD) is a rare monoclonal gammopathy that involves the deposition of light chains (LC) in multiple organs, leading to progressive dysfunction. The kidney is usually the most affected organ and responsible for the initial clinical manifestations. We present the case of a patient with LCDD with prominent liver involvement (marked cholestasis, hepatomegaly and portal hypertension) but with no evidence of coexisting lymphoproliferative disorder. LEARNING POINTS Light-chain deposition disease (LCDD) is rare, and diagnosis can be challenging, especially in the absence of a coexistent lymphoproliferative disorder. The presence of light chains in blood or urine can be intermittent, and immunohistochemistry results of biopsy can be difficult to interpret. Hence, there is a need for a high level of suspicion in this diagnosis. Although renal involvement is the most classic presentation, LCDD should be suspected when there is multiple organ involvement, as in this case, even when renal manifestations do not dominate the clinical picture. Prognosis depends on the affected organs, and treatment should be started as rapidly as possible to suppress production of the clonal light-chain and halt organ damage.

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“…We performed a transjugular biopsy, as a percutaneous biopsy was contraindicated, because of the elevated prothrombin time (INR 1, 30). Moreover, a percutaneous biopsy could carry an increased risk of bleeding, which may possibly lead to hepatic failure [ 2 , 9 , 10 ].…”

Section: Discussionmentioning

confidence: 99%

Primary Amyloidosis Manifesting as Cholestatic Jaundice after Laparoscopic Cholecystectomy

Misiakos

Bagias

Tiniakos

et al. 2015

Case Reports in Surgery

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A 71-year-old female patient with cholelithiasis who had undergone laparoscopic cholecystectomy was admitted with obstructive jaundice (total bilirubin ~6 mg/dL) three months later. An ERCP was performed, in which a gallstone was found, followed by a sphincterotomy and cleansing of the bile duct. Due to deterioration of jaundice (>25 mg/dL), a new, unsuccessful ERCP and stent placement was carried out. Because of ongoing cardiac failure, she underwent an echocardiogram which revealed restrictive cardiomyopathy possibly due to amyloidosis. A liver biopsy was performed, which was positive for amyloid deposits in the liver, and the diagnosis was confirmed by the detection of monoclonal λ IgG protein in urine. The patient's jaundice gradually deteriorated and she died one week later from hepatic insufficiency.

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Light Chain Deposition Disease Presenting as Cholestatic Jaundice: A Case Report

Cited by 8 publications

References 8 publications

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

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

Light-Chain Deposition Disease with Prominent Hepatic Involvement

Primary Amyloidosis Manifesting as Cholestatic Jaundice after Laparoscopic Cholecystectomy

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