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 ...

We report on a woman with hepatic involvement of primary systemic (immunoglobulin light chain, AL) amyloidosis. Her diagnosis was confirmed by liver biopsy. Clinical symptoms of hepatic amyloidosis are generally mild at its first stage, with most frequent findings being hepatomegaly and alkaline phosphatase elevation. Recent advances in the understanding of the pathophysiology of systemic amylo...

BACKGROUND Primary immunoglobulin light chain amyloidosis (AL amyloidosis) is a plasma cell disorder which mainly affects heart, kidneys, liver, and peripheral nervous system. Cases of atypical AL amyloidosis presented as spontaneous vertebral compression fractures have been rarely reported, and data about the management and clinical outcomes of the patients are scarce. METHODS Herein, we pre...

Liver is one of the most commonly involved organs in both primary and secondary systemic amyloidoses, but hepatic amyloidosis, manifested as mild to moderate enlargement, is usually not symptomatic nor it is clinically problematic. Rarely, massive hepatomegaly, severe cholestatic hepatitis or liver failure may be encountered in patients with systemic amyloidosis. Two cases with lambda light-cha...

For several years, there has been a significant increase in the number of patients diagnosed with cardiac amyloidosis. However, are still difficulties associated diagnosis, identifying its type and further treatment disease. The diversity clinical symptoms, absence pathognomonic symptoms make it difficult to diagnose, which leads progression Heart involvement is main cause morbidity mortality s...

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 ...

• 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...

In 1854, the term "amyloid" was first used in the description of a liver specimen at autopsy by Virchow. The kidneys and heart are the most commonly involved organs in amyloidosis; liver and gastrointestinal tract involvement is less common, and the symptoms are usually mild. Here, we report the case of a 57-year-old male patient who presented with oral hemorrhagic bullae, thrombocytopenia and ...

BACKGROUND Spontaneous hepatic bleeding is a rare but potentially life-threatening complication of primary systemic amyloidosis. Although the liver is a common site of amyloid deposition, clinical presentation is usually mild or absent. CASE We report a case of a female patient, who had been repeatedly surgically revised because of liver rupture and hemoperitoneum. Initially, the computed tom...

Globular amyloidosis is a very infrequent amyloidosis subtype, characterized by the deposition of rounded bodies of protein, occasionally perivascular instead of the usual linear deposits. The most frequently affected organ is the liver, although other organs can also been involved. To date, only eight cases (1-3) of globular amyloidosis of the gastrointestinal tract have been described. Clinic...