Liver Failure Caused by Light Chain Deposition Disease Associated with Multiple Myeloma

Mena-Duran, Armando V.; Vicente, Elia Muñoz; Llorens, Gonzalo Pareja; Cervera, José Sanchis

doi:10.2169/internalmedicine.51.6510

Cited by 8 publications

(7 citation statements)

References 27 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 ]. As seen with other causes of liver failure, the clinical presentation in these patients is generally encephalopathy, coagulopathy, and jaundice.…”

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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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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“…Only a few case reports in the past few years have been able to successfully touch on the rarity of our patient's presentation of acute liver failure in the face of newly diagnosed amyloidosis and MM [12][13][14][15]. More importantly, these cases have highlighted the rapid decline associated with acute liver failure in these patients.…”

Section: Discussionmentioning

confidence: 99%

Amyloidosis presenting as acute liver failure: a case report

Parikh¹,

Tsai²

2014

Intern Med Inside

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Introduction: Amyloidosis has two principle types: multiple myeloma-associated (AL) and amyloidassociated amyloidosis (AA). Both types can be local or systemic and can often involve the liver. Hepatic amyloidosis carries a poor prognosis, but commonly presents with a more gradual time course of evolving hepatomegaly and elevated serum alkaline phosphatase. This casereport involves an unusual presentation of hepatic amyloidosis with acute liver decompensation resulting in death. Case presentation: A 76 year old man presented with complaints of progressive weakness and anorexia resulting in weight loss. He was initially found to have laboratory abnormalities notable for cholestasis and a mild transaminitis. On exam, the patient was cachectic with scleral icterus, jaundice, and abdominal distention. Liver ultrasound with doppler imaging was significant for increased echogenicity of the liver and ascites. The patient underwent transjugular liver biopsy, which demonstrated hepatic amyloid. Additional testing revealed a diagnosis of amyloidosis secondary to multiple myeloma. On day 5 of the patient's admission, cholestasis and transaminitis acutely worsened, with eventual peaking of aspartate aminotransferase to 3362 u/l, alanine aminotransferase to 1439 u/l, and total bilirubin to 18.8 mg/d. Further work-up for this acute decompensation, including imaging and laboratory tests, did not reveal any triggering events such as portal vein thrombosis or intra-abdominal bleeding from recent liver biopsy. Additionally, cardiovascular compromise, infectious etiologies, and toxins were ruled out. Unfortunately, the patient was not a candidate for chemotherapy given his acute hepatic decompensation and subsequent encephalopathy on day 8 of admission. The patient was started on Lactulose and N-acetylcysteine, though his mental status continued to worsen and the patient died on day 10 of admission from multi-organ compromise secondary to acute liver failure. Conclusion: Amyloidosis resulting in acute hepatic failure has previously been reported but remains quite rare. Clinicians must consider infiltrative diseases, such as multiple myeloma and amyloidosis, when patients present with an extended prodromal illness paired with unexplained, worsening hepatic dysfunction. Likewise, physicians should be aware that patients who present with hepatic amyloid secondary to multiple myeloma are at high risk for rapid clinical deterioration.

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“…Less frequently, LCDD may occur in the context of lymphoid hemopathy such as Waldenström disease. Unlike AL amyloidosis, LC deposits in LCDD, mostly the kappa isotype, do not stain with Congo red, and show a typical histological linear pattern along basement membranes on immunofluorescence microscopy, with a characteristic linear powdery punctate appearance on electron microscopy [10, 11].…”

Section: Discussionmentioning

confidence: 99%

An Unusual Case of Cholestatic Hepatitis due to Light-Chain Deposition Disease

Grembiale¹,

Garlatti²,

Ermacora³

et al. 2020

Case Rep Oncol

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Light-chain deposition disease (LCDD) is a rare paraproteinaemia characterized by the deposition of monoclonal immunoglobulins with a non-fibrillar structure and hence Congo red negative deposits. Kidney disease is the more frequent manifestation, but other organs may also be involved. A 70-year-old man with hypertension and mild chronic renal failure showed a hepatomegaly without splenomegaly. His renal and liver test rapidly got worse. A serum electrophoresis and immunofixation isolated monoclonal kappa light-chain gammopathy, with serum free kappa light chain excess. The bone marrow biopsy showed the presence of interstitial infiltration of plasma cells like multiple myeloma type at initial phase. Periumbilical fat biopsy was negative. Echocardiography demonstrated an infiltrative cardiac disease. The biopsies of the duodenum small intestine mucosa showed flaps with eosinophil material (Masson’s staining) with atrophic crypts and chronic inflammation at chorion level. Amyloid substance was negative. There was a strong positivity for light chains kappa compatible with LCDD. A liver biopsy confirmed this finding. Therapy with dexamethasone and bortezomib improved clinical state and hepatic and renal laboratory tests. Chemotherapy based on novel anti-myeloma agents should be rapidly considered in LCDD patients with severe organ involvement.

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Liver Failure Caused by Light Chain Deposition Disease Associated with Multiple Myeloma

Cited by 8 publications

References 27 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

Amyloidosis presenting as acute liver failure: a case report

An Unusual Case of Cholestatic Hepatitis due to Light-Chain Deposition Disease

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