amyloidosis is particularly difficult to diagnose because the signs and symptoms are subtle . additionally , there are no specific imaging or laboratory tests , except histopathology .
although it is considered to be a systemic disorder , a small portion of cases may be localized .
a 54-year - old man presented with nonspecific symptoms ( jaundice and back pruritus ) .
biochemical tests showed a high level of bilirubin and elevated serum tumor markers ( ca199 and ca125 ) .
amyloidoses are a group of disorders that primarily consist of 3 forms , namely primary amyloidosis , secondary amyloidosis , and familial amyloidosis . amyloidosis is characterized by the deposition of abnormal proteins undergoing a conformational change to form insoluble -sheet protofilaments .
the difficulty associated with diagnosing amyloidosis relates to the lack of specific imaging or laboratory tests , which poses a great challenge for clinicians .
cases of amyloidosis that are primarily localized in the liver rarely have been reported . here
, we report a case involving a patient with primary hepatic amyloidosis who presented with liver dysfunction .
a 54-year - old man was admitted to our hospital for gradual jaundice and weight loss over 2 months .
his vital signs were stable and afebrile . on physical examination , his skin and sclera were mildly jaundiced , with scratch marks on his arms and back .
biochemical tests showed a high level of both total and direct bilirubin ( 97.8  mol / l and 82.6  mol / l , respectively ) .
serum alkaline phosphatase ( alp ) ( 374  /l ) and gamma glutamyl transpeptidase levels ( -gtt ) ( 319  /l ) were also elevated ( listed in table 1 ) .
computed tomography ( ct ) and magnetic resonance ( mr ) imaging showed hepatomegaly with no suspicious nodules ( fig .
1 ) . to determine whether there were blockages in the bile ducts , magnetic resonance cholangiopancreatography ( mrcp ) was used and showed hepatomegaly with cholecystitis .
he then was treated with ursodeoxycholic acid capsules and compound ammonium glycyrrhetate single s and ademetionine for a week .
after consent was obtained from the patient , ultrasonography - guided liver biopsy was utilized .
the liver biopsy demonstrated a massive amount of amyloid deposition along the sinusoids ( fig . 2c 
( a ) a biopsy specimen from the skin , photomicrograph ( h&e stain ) showed collagen - type extracellular material , ( b ) characteristic  apple - green  birefringence on polarized light microscopy , which is consistent with amyloidosis , ( c ) biopsy specimen from the liver tissue , photomicrograph ( h&e stain ) showed collagen - type extracellular material , ( d ) congo red stain demonstrated characteristic staining , ( e ) methyl violet stain revealed strong affinity for the material , ( f ) immunostaining with antibodies to kappa light chains was negative , ( g ) immunostaining with antibodies to lambda light chains was positive , ( h ) the biopsy specimen from the skin , immunofluorescence with antibodies to lambda light chains was positive .
since the patient was found to have amyloid deposition in his liver , we had to conduct other tests to determine whether it was localized or systemic .
echocardiographic and renal function tests , bone marrow aspiration , serum - free light chain test , and skin biopsy ( from multiple sites ) were performed .
histopathological evaluation showed positive staining with congo red and characteristic  apple - green  birefringence on polarized microscopy .
all these results indicated primary hepatic amyloidosis , and the skin biopsy was positive for lambda light chains ( fig .
serum bilirubin and amino transaminases levels measured at admission and afterward are listed in table 2 .
the patient refused to take melphalan or undergo stem cell transplant ; therefore , we provided him with supportive therapies .
subsequently , he was discharged from the hospital and continued to take oral chemotherapy ( thalidomide 100  mg / d and prednisone 20  mg / w ) .
immunoglobulin light chain amyloidosis is a clonal plasma cell disorder in which amyloid fibrils that are derived from immunoglobulin light chains are deposited in organs and tissues .
the prognosis is often poor if fibril deposits build up in vital organs such as cardiac muscles . with no treatment ,
the median survival time is 2 years . as reported , a patient with primary amyloidosis presented symptoms that were similar to those of crohn 's disease , delaying accurate diagnosis .
since the presenting symptoms tend to mimic those of other diseases , physicians should suspect amyloidosis in any patient with generalized fatigue , weight loss , skin lesions , paresthesias , nondiabetic nephrotic syndrome , and hepatomegaly .
amyloidosis is usually considered to be systemic , but 10% to 20% of cases can be localized . here , we reported a case in which a patient presented with jaundice and altered liver function tests . at first , the elevated serum tumor markers and enlarged liver suggested liver cancer or carcinoma of the gall bladder .
however , imaging results did not support cancer . since the patient was allergic to iodine , contrast - enhanced ct was forbidden .
the diagnosis of amyloidosis requires 2 components : a generic and a type - specific diagnosis .
invasive organ biopsy is not always necessary , as fat pad aspiration , in particular , is even more sensitive ( 72% ) than bone marrow biopsy in diagnosing amyloidosis .
however , if a fat pad aspiration fails to demonstrate amyloidosis , then biopsies of involved organs should be considered .
currently , mass spectrometry - based proteomics have emerged as a new technique to classify systemic amyloidosis and analyze serum transthyretin in patients with potentially amyloidogenic mutations .
serum - free light chains , protein immunofixation , mr elastographic , shear - wave elastography , and other examinations support the diagnosis of amyloidosis , but they are not sufficient to define it . a proposed strategy for evaluating a patient with suspected amyloidosis
treatment for amyloidosis depends on the type of amyloidogenic protein . for amyloid light - chain ( al )
it has been reported that melphalan and prednisone are superior to colchicine in 2 randomized phase iii studies .
melphalan in combination with dexamethasone is highly effective in treating al amyloidosis with minimal toxicity .
autologous stem cell transplantation is a reasonable primary option , but only 20% of patients meet the requirements .
researchers have explored new drugs ( thalidomide , lenalidomide , pomalidomide , and bortezomib ) to treat al amyloidosis in combination with dexamethasone or melphalan and dexamethasone or dexamethasone and cyclophosphamide .
melphalan combined with dexamethasone is still considered to be the standard treatment for nontransplant candidates . however , more data are required on treatment options in association with the pathophysiology of the amyloid formation that is generated .
amyloidosis is uncommon , and its prognosis is poor , especially when irreversible organ damage occurs .
thus , the most urgent requirement is an early and accurate diagnosis of the disease .
clinicians should be aware of all the possible signs and initiate an effective treatment as early as possible .
 abstractbackground : amyloidosis is particularly difficult to diagnose because the signs and symptoms are subtle . additionally , there are no specific imaging or laboratory tests , except histopathology . 
 although it is considered to be a systemic disorder , a small portion of cases may be localized.introduction of the case : a 54-year - old man presented with nonspecific symptoms ( jaundice and back pruritus ) . biochemical tests showed a high level of bilirubin and elevated serum tumor markers ( ca199 and ca125 ) . 
 routine imaging showed hepatomegaly without heterogeneous enhancement . 
 liver biopsy confirmed the diagnosis of hepatic amyloidosis . 
 no cardiac or renal involvement was found . 
 the patient accepted treatment involving oral chemotherapy.conclusion:a rare and unique presentation of hepatic amyloidosis was highlighted in this case . 
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Introduction
Case report
Discussion