Portal hypertension and acute liver failure as uncommon manifestations of primary amyloidosis

Norero, Blanca; Pérez-Ayuso, Rosa María; Duarte, Ignacio; Ramírez, Pablo; Soza, Alejandro; Arrese, Marco; Benítez, Carlos

doi:10.1016/s1665-2681(19)30916-0

Cited by 17 publications

(7 citation statements)

References 28 publications

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“…The cardiac manifestations are often well‐characterized, while systemic vascular amyloidosis seems to represent an under‐recognized condition. Occasionally, amyloid angiopathy has been reported to cause, for example, ocular problems in familial TTR amyloidosis or pulmonary hypertension in AL amyloidosis . Furthermore, systematic studies in AL patients, who often have widespread vascular amyloidosis, revealed significant vascular and endothelial dysfunction .…”

Section: Discussionmentioning

confidence: 99%

Gelsolin amyloid angiopathy causes severe disruption of the arterial wall

Koskelainen

Pihlamaa

Suominen

et al. 2016

APMIS

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Hereditary gelsolin amyloidosis (HGA) is a dominantly inherited systemic disease reported worldwide. HGA is characterized by ophthalmological, neurological, and dermatological manifestations. AGel amyloid accumulates at basal lamina of epithelial and muscle cells, thus amyloid angiopathy is encountered in nearly every organ. HGA patients have cardiovascular, hemorrhagic, and potentially vascularly induced neurological problems. To clarify pathomechanisms of AGel angiopathy, we performed histological, immunohistochemical, and electron microscopic analyses on facial temporal artery branches from 8 HGA patients and 13 control subjects. We demonstrate major pathological changes in arteries: disruption of the tunica media, disorganization of vascular smooth muscle cells, and accumulation of AGel fibrils in arterial walls, where they associate with the lamina elastica interna, which becomes fragmented and diminished. We also provide evidence of abnormal accumulation and localization of collagen types I and III and an increase of collagen type I degradation product in the tunica media. Vascular smooth muscle cells appear to be morphologically and semi-quantitatively normal, only their basal lamina is often thickened. In conclusion, angiopathy in HGA results in severe disruption of arterial walls, characterized by prominent AGel deposition, collagen derangement and severe elastolysis, and it may be responsible for several, particularly hemorrhagic, disease manifestations in HGA.

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Section: Discussionmentioning

confidence: 99%

Gelsolin amyloid angiopathy causes severe disruption of the arterial wall

Koskelainen

Pihlamaa

Suominen

et al. 2016

APMIS

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“…Hepatic dysfunction is usually clinically silent with normal or mildly abnormal liver function tests [8]. A few cases of acute or subacute hepatic failure have previously been reported in AL amyloidosis with multiple myeloma [8][9][10][11][12][13][14][15][16][17].…”

Section: Discussionmentioning

confidence: 99%

Systemic AL amyloidosis presenting as progressive hepatic failure with coagulopathy: An autopsy case report

Xu¹,

Ward²

2020

Int J Case Rep Images

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Introduction: Systemic AL amyloidosis is defined as extracellular deposition of insoluble immunoglobulin light chains, amyloid fibril protein AL, in at least one vital organ.Here we report a case of systemic AL amyloidosis presenting as progressive hepatic failure with coagulopathy. Case Report:The patient was a 62-year-old African American female first admitted to our hospital due to jaundice, easy bruising, fatigue, and weight loss for three months. There were mild abnormal liver function tests with coagulopathy, polyclonal gammopathy, and mild hepatomegaly by imaging. She was discharged after correction of coagulopathy. She presented with altered mental status one month later. Lab results showed similar abnormal liver function tests and hepatomegaly but with severe coagulopathy. The patient was found to have oozing blood, severe hypoxia, and expired the next day. A complete autopsy revealed systemic amyloidosis microscopically, and confirmed by Congo red stain. The liver demonstrated the most extensive deposition of amyloid. Liquid chromatography tandem mass spectrometry revealed AL (kappa)-type amyloid deposition. Bone marrow showed kappa light chain predominance. Conclusion: Systemic amyloidosis presenting as progressive hepatic failure is very rare. Amyloidosis with liver involvement is one of the important differential diagnoses even when patients present with mild liver dysfunction (especially elevated alkaline phosphatase and gamma-glutamyl transerase) and coagulopathy.

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“…In an autopsy series [ 4 ], 70% of the patients with primary systemic amyloidosis had liver involvement, which is, however, most of the times asymptomatic. There are few reported cases of portal hypertension due to primary systemic amyloidosis [ 5 - 6 ]. Notably, liver involvement is associated with a reduced survival in patients with primary systemic amyloidosis (mean survival time: 10 to 14 months vs. 12 to 18 months in patients without liver involvement) [ 7 - 9 ] and the presence of portal hypertension further worsens patients’ outcome (mean survival time: eight to nine months) [ 10 ].…”

Section: Discussionmentioning

confidence: 99%

Portal Hypertension and a Stiff Liver

D’Alitto

Scherz

Margini

et al. 2018

Cureus

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Portal hypertension (PH) is a common clinical syndrome leading to severe complications. In the western world, about 90% of cases of PH are due to liver cirrhosis, and thanks to the availability of ultrasound elastography methods, this diagnosis is usually confirmed at bedside. We report a case of a patient presenting with PH and ascites initially suspected of suffering from liver cirrhosis. The finding of large hepatomegaly and a massive increase in liver stiffness prompted us to perform a liver biopsy. This revealed no fibrosis, but diffuse primary amyloidosis (AL amyloidosis). We discuss the diagnostic and treatment of this case, with emphasis on non-invasive imaging methods available for diagnosis and follow up.

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Portal hypertension and acute liver failure as uncommon manifestations of primary amyloidosis

Cited by 17 publications

References 28 publications

Gelsolin amyloid angiopathy causes severe disruption of the arterial wall

Gelsolin amyloid angiopathy causes severe disruption of the arterial wall

Systemic AL amyloidosis presenting as progressive hepatic failure with coagulopathy: An autopsy case report

Portal Hypertension and a Stiff Liver

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