Spontaneous Hepatic Rupture in Amyloidosis – Report of a Case

Lehmann, Thorsten G.; Scheble, Veit; Miller, Stephan; Heininger, Alexandra; Fend, Falko; Koenigsrainer, Alfred

doi:10.1055/s-0032-1325340

Cited by 7 publications

(8 citation statements)

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“…There are speculations that amyloid deposits make the liver capsule stiff and easier to break at the same time as the liver itself gets larger because of swelling or bleeding. In addition, amyloid deposits in the vessel walls may make the vessels fragile [6][7]. Moreover, deficiencies of coagulation factors, especially factor X, as well as well hypofibrinogenemia may contribute to bleeding [5][6].…”

Section: Discussionmentioning

confidence: 99%

“…Traditionally emergency laparotomy with packing and sometimes hepatic artery ligation has been performed in the event of liver rupture, although transarterial embolization has become an emerging treatment option during the recent decade. In a case report Lehmann et al suggest transarterial embolization to be first choice to manage spontaneous liver rupture [7]. In six of the cases laparotomy was carried out with mixed outcomes, four cases resulting in death and two cases resulting in recovery.…”

Section: Discussionmentioning

confidence: 99%

“…Systemic AL amyloidosis may manifest with cardiac, kidney, neurological, soft tissue as well as gastrointestinal and hepatic involvement [1,4]. Structural changes in the liver and hepatic vessels in combination with a variety of coagulopathies may contribute to spontaneous liver rupture and bleeding in amyloidosis [1,[5][6][7]. Although hepatic involvement in amyloidosis is common, liver rupture or bleeding is a rare entity.…”

Section: Introductionmentioning

confidence: 99%

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Amyloidosis and Spontaneous Liver Bleeding: A Case Report and Literature Review

Huang¹

2019

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Liver rupture with severe liver bleeding in patients with amyloidosis is an unusual but generally difficult to deal with and potentially fatal complication in these patients. Even patients with known manifestations to the lever can present with acute lever bleeding. Here we present a case report of a 62-year-old male with systemic immunoglobulin light chain (AL) amyloidosis, known manifestations to the lever and spontaneous liver bleeding which was treated conservatively without the need of surgical intervention. The etiology, panorama of clinical manifestations of hepatic amyloidosis and the management of liver bleeding in association with this unusual disease are reviewed. 18 cases of hepatic rupture and haemorrhage in association with amyloidosis in previously published cases are summarized. The choice of management should be individualized and the patient´s hemodynamic status and extent of liver bleeding are important factors to be taken into consideration. In all patients it is crucial to exclude underlying malignancy as the reason of bleeding. The patient´s hemodynamic status and the extent of liver bleeding are the most important factors to be taken into consideration in order to establish individualized management plan. Increased awareness of severe liver bleeding from amyloidosis may achieve reduced mortality of this potentially fatal complication.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Amyloidosis and Spontaneous Liver Bleeding: A Case Report and Literature Review

Huang¹

2019

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“…In fact, recent research has emphasized the increased risk of bleeding from liver biopsy in patients with amyloid deposition though the transjugular approach has decreased such risks when compared to the percutaneous method [23,24]. Additionally, portal hypertension in the setting of hepatic amyloidosis can lead to severe complications, including subcapsular hematoma and spontaneous rupture of the liver [25,26]. While the above possibilities were thoroughly considered as the source of our patient's decompensation, the work-up during the transition to acute liver failure remained unrevealing.…”

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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“…À notre connaissance, l'association amylose hépatique primitive AL et syndrome hépatorénal n'a pas encore été rapportée à ce jour. Les rares complications hépatiques décrites sont les ruptures hémorragiques spontanées, l'insuffisance hépatocellulaire aiguë et l'hypertension portale [3,7,8].…”

Section: Observationunclassified