despite the fact that hepatic involvement is frequently seen in systemic primary amyloidosis, major hepatic symptoms as primary manifestation and severe impaired liver function are rare. herein, we report a 38-year-old woman with primary hepatic amyloidosis, and severe  portal hypertension . the patient had ascites and markedly elevated alkaline phosphatase level at presentation. she had a rapi...

• Leukocyte cell-derived chemotaxin-2–associated amyloidosis (ALect2) is a common cause of systemic amyloidosis involving the liver. • Recognition and accurate diagnosis of hepatic ALect2 amyloidosis is essential for accurate management of patients with hepatic amyloidosis. Using laser microdissection and mass spectrometry (MS)-based proteomics, we subtyped amyloid deposits from 130 cases of he...

Hyperimmunization of horses with specific antigens to harvest antisera is associated with high incidence of liver amyloidosis. The histopathologic examination is the gold standard method for diagnosis of the hepatic amyloidosis. The aim of the present study was to determine whether the clinical manifestations and serum values of appropriate chemical constituents due to liver injury, provide the...

Amyloidosis is a rare disorder with a wide spectrum of presentations and anomalies. It is subdivided into 2 broad categories based on protein deposition; primary and secondary amyloidosis. It can present as a single-organ involvement or as a diffuse infiltrative multi-organ process. Isolated hepatic amyloidosis presentation is a rare phenomenon that develops due to insoluble amyloid deposition ...

BACKGROUND AND AIMS The liver is frequently involved in amyloidosis but the significance of hepatic amyloid has not been systematically studied. We have previously developed scintigraphy with 123I serum amyloid P component (123I-SAP) to identify and monitor amyloid deposits quantitatively in vivo and we report here our findings in hepatic amyloidosis. METHODS Between 1988 and 1995, 805 patien...

Hepatic involvement of amyloidosis is common. Diffuse infiltration with hepatomegaly is a usual radiologic finding of hepatic amyloidosis. To our knowledge, this is the first case of amyloidosis involving the liver that presented as a mass.

Hepatic amyloidosis is a rare disease entity that results from insoluble amyloid protein deposition in the liver. The disease often presents with vague, nonspecific clinical features. Currently, there is little literature describing treatment outcomes for biopsy-proven hepatic amyloidosis and current treatment guidelines recommend that patients enroll in a clinical trial due to insufficient evi...

Although systemic amyloidosis of amyloid-associated protein (AA) type (secondary or reactive amyloidosis) frequently involves the liver, it rarely causes clinically apparent liver disease. Mild elevation of alkaline phosphatase and hepatomegaly are the most common biochemical and clinical findings, respectively. We report a case of systemic amyloidosis of AA type, which clinically presented as ...

The clinically reported case of liver involvement with multiple myeloma (MM) is rare. Amyloidosis, defined as a tissue deposition of clonal light-chain fibrils, has been reported in 10-15% of the MM patients. We described a rare MM patient with the primal presentation of fulminant hepatic failure and biliary system involvement due to amyloidosis. Our patient had the primal symptoms of hyperbili...

CONTEXT Amyloidosis is a disease of extracellular deposition of misfolded proteinaceous subunits, which could be systemic or localized disease. Though hepatic amyloidosis was not uncommon in autopsy series, most cases of hepatic amyloidosis were asymptomatic. Ascites, jaundice, portal hypertension, and gastrointestinal bleeding from esophageal varices were reported in literature. CASE REPORT ...