Hepatic Amyloidosis Research Articles

Leukocyte cell-derived chemotaxin 2 (LECT2)–associated amyloidosis is a frequent cause of hepatic amyloidosis in the United States

AbstractUsing laser microdissection and mass spectrometry (MS)-based proteomics, we subtyped amyloid deposits from 130 cases of hepatic amyloidosis. Although we confirmed that immunoglobulin light chain amyloidosis was the most frequent cause of hepatic amyloidosis, leukocyte cell-derived chemotaxin 2 (LECT2) amyloidosis (ALect2) accounted for 25% of cases. This novel finding was associated with Hispanic ancestry, incidental discovery of amyloid in liver specimens sampled for other unrelated conditions, and a characteristic pattern of hepatic amyloid deposition. Although ALect2 patients had a common LECT2 polymorphism, pathogenic mutations were not discovered, suggesting that constitutive or compensatory LECT2 overexpression led to ALect2 deposition. These findings indicate that ALect2 is a common cause of hepatic amyloidosis in the population of the United States, and subtyping hepatic amyloid deposits by an accurate analytic method such as MS is required for optimal clinical management of hepatic amyloidosis patients and to avoid incorrect and unnecessarily toxic therapies.

Hepatic ALECT-2 Amyloidosis Causing Portal Hypertension and Recurrent Variceal Bleeding

To examine laser microdissection and mass spectrometry (LMD-MS), which has emerged as a new tool to aid in typing amyloid proteins. ALECT-2 is a potential cause of hepatic amyloidosis best detected by LMD-MS. One of the more recently reported proteins is ALECT-2 (leukocyte cell-derived chemotaxin 2) amyloid, found in renal specimens of Hispanic patients. Here we report the first case of hepatic ALECT-2 amyloidosis diagnosed by LMD-MS from a liver biopsy specimen of a 52-year-old Hispanic man and causing portal hypertension with recurrent esophageal variceal bleeding. ALECT-2 can cause amyloidosis in organs other than the kidneys. It should be strongly considered in Hispanic patients and in those with a globular pattern of amyloid deposition. The incidence of ALECT-2 amyloidosis is likely underreported.

Primary Hepatic Amyloidosis: An Untimely Death

Purpose: Primary hepatic amyloidosis, also termed hepatic AL amyloidosis, is characterized by the deposition of amyloid fibril-forming monoclonal immunoglobulin light chains within the liver. This disease often starts with mild signs, such as hepatomegaly and elevated alkaline phosphatase. Over time, this progresses to liver dysfunction, ascites, portal hypertension, liver failure, and death. A 63-year-old African-American female presented to the hospital with a one-month history of abdominal distension and jaundice. Her physical findings included ascites and massive hepatomegaly, and labs showed an elevated alkaline phosphatase (ALP) 1989 U/L, total bilirubin 4.8 mg/dL, aspartate aminotransferase (AST) 92 U/L, alanine aminotransferase (ALT) 43 U/L, platelet (PLT) 750 K/uL, prothrombin time (PT) 24.5 seconds, INR 2.2, total protein 5.5 g/dL, albumin 2.6 g/dL, blood urea nitrogen (BUN) 11 mg/dL and creatinine (Cr) 0.4 mg/dL. A CT scan of the abdomen revealed hepatomegaly with an echogenic lesion in the right hepatic lobe, splenomegaly, and ascites. A liver biopsy was done, showing Congo red stain with apple-green birefringence consistent with amyloidosis. Her bone marrow biopsy showed amyloidosis with aberrant kappa-restricted plasma cell population. She was diagnosed with hepatic AL amyloidosis, and she was started on bortezomib and dexamethasone. However, one month later, she had an elevated ALP 3274 U/L, total bilirubin 26.7 mg/dL, AST 101 U/L, ALT 57 U/L, PLT 357 K/uL, PT 35.8 seconds, INR 3.5, and Cr 3.5 mg/dL. She had a renal ultrasound that showed an increased echogenicity of both kidneys. Given her poor performance status, she did not receive dialysis or further chemotherapy. She was sent home with hospice, and passed away less than one month later. Prognosis is poor in patients with hepatic amyloidosis, with a mean survival of 10-14 months from diagnosis. This disease usually presents with mild clinical findings; however, once liver failure develops, the survival rate declines rapidly. Although this disease has a poor prognosis, early diagnosis can lead to more effective therapy.

Fatal Bleeding Due to Acquired Factor IX and X Deficiency: A Rare Complication of Hepatic AL Amyloidosis

Purpose: Systemic AL amyloidosis is a disorder of progressive extracellular deposition of insoluble fibrils derived from λ/κ Ig light chains within various organs. Hepatic deposition (clinically silent) occurs in half of cases and liver is rarely the dominant organ. Often, bleeding in AL amyloidosis is multifactorial, including vessel fragility from amyloid infiltration, hyper fibrinolysis, platelet dysfunction and uremia. We report a case of amyloidosis with uncontrolled hemorrhage due to a severe acquired combined factors IX and X deficiency. Methods: A previously healthy 62-year-old man presented with acute abdominal pain with history of vague abdominal discomfort, fatigue, bruisability and microscopic hematuria for two months, but no findings to suggest nephrolithiasis or cancer were identified on imaging and cystoscopy. He was hemodynamically stable, exam was notable for some bruising, abdominal distention and tenderness, but no guarding or heptosplenomegaly. Results: Labs including CBC, electrolytes, LFTs, fibrinogen, thrombin time and D-dimer were normal. PT, INR, and PTT were elevated, but mixing studies were normal. Coagulation factor IX and X levels were noted to be deficient. Free λ light chain was elevated, corresponding to a monoclonal IgG λ band in the γ region, while free κ light chain and β2 microglobulin were normal. With the absence of personal and family history of liver disease, bleeding disorder and subsequent rise in alkaline phosphatase without biliary obstruction, it was concerning for an intrinsic liver disease. With acquired combined factor deficiency in the setting of MGUS, amyloidosis was considered. Liver biopsy was deferred given the concern for increased bleeding risk and hepatic rupture. He died of GI and intra-peritoneal bleeding, despite multiple transfusions including factors, FIEBA. Autopsy showed massive deposition of amyloid within liver and spleen, due to plasma cell dyscrasia with monotypic λ staining plasma cells in bone marrow accounting for 15% of nucleated cell forms. Conclusion: Delayed diagnosis is common in AL amyloidosis due to nonspecific clinical presentations, as in our case. Hepatic amyloidosis carries a poor prognosis, but has no specific effective treatment. Liver transplantation may be a life-saving procedure in selected cases of rapidly progressing hepatic amyloidosis.Figure: Liver section showing sinusoids distended by pink staining amyloid.

An Unusual Case of Rapidly Progressive Hepatic Failure and Death due to Primary Hepatic AL (kappa) Amyloidosis

Purpose: Hepatic amyloidosis is a rare infiltrative disease involving the liver. Although hepatic involvement is common in systemic amyloidosis, clinically significant liver failure is very rare. This is an unusual case of a 62-year-old man with past medical history of DM Type II, HTN, and chronic renal insufficiency, who initially presented with symptoms of weight gain, abdominal distension, altered mental status, worsening fatigue, and diarrhea over a period of three weeks. He was found to have hepatomegaly and ascites on exam, while lab studies showed evidence of acute renal failure, severe anemia, and coagulopathy. An ultrasound of the abdomen showed evidence of ascites and hepatosplenomegaly, while EGD showed evidence of small esophageal varices and portal hypertensive gastropathy. A biopsy of the liver was performed for further evaluation, which showed atrophic hepatocytes with severe sinusoidal deposition of homogenous eosinophilic material, with a positive congo-red stain showing apple green birefringence consistent with amyloid deposits. The sample was further sent for liquid chromatography tandem mass spectrometry, which detected a peptide profile consistent with AL (kappa) amyloid deposition. His hospital course became complicated with oral and gastric mucosal bleeding refractory to replacement of coagulation factors. Also, complications of dialysis-dependent renal failure, hypoxic respiratory failure, and gram negative sepsis further contributed to his declining condition. He died soon after from hypovolemic shock secondary to ongoing mucosal bleeding refractory to treatment. Patients with primary systemic amyloidosis who have biopsy-proven liver involvement (primary hepatic amyloidosis) have a poor prognosis; the median survival of patients has been reported as 8.5 months, while in this case, he rapidly deteriorated within six weeks of being diagnosed for amyloidosis.

A case of fatal intrahepatic cholestasis with primary AL amyloidosis: is early diagnosis possible?

Immunoglobulin light chain-associated (AL) amyloidosis is a multisystemic disorder characterized by extracellular deposition of immunoglobulin light chain produced by a proliferative plasma cell clone. Although the liver is the major organ involved in AL amyloidosis, hepatic involvement is often clinically asymptomatic and severe intrahepatic cholestasis as the primary manifestation of the disease is rare. A 60-year-old man with severe jaundice, massive ascites and highly elevated alkaline phosphatase was diagnosed with AL amyloidosis by a transjugular liver biopsy. He had undergone a yearly medical check that showed no abnormalities except for mild elevation of serum γ-glutamyltransferase at 1year before admission. Owing to his poor condition and rapidly progressive liver and renal dysfunction, neither stem cell transplantation nor a combination of chemotherapeutic agents could be applied, and he died 1.5months after admission. An autopsy revealed amyloid deposition in the systemic organs, and there was no evidence of multiple myeloma. Continuous elevation of γ-glutamyltransferase may be a useful marker for early diagnosis of fatal hepatic amyloidosis.

Two cases with hepatic amyloidosis suspected of having primary sclerosing cholangitis

Sclerogenic biliary changes in hepatic amyloidosis are seldom observed. Here, we report two recent cases initially suspected as primary sclerosing cholangitis (PSC), which were later diagnosed as hepatic amyloidosis (AL type). Case 1: On the basis of magnetic resonance cholangiopancreatography (MRCP) findings, PSC was suspected in a 41-year-old woman with jaundice. Computed tomography (CT) showed nodular pulmonary lesions and swollen cervical, mediastinal and para-aortic lymph nodes, the cause of which was unknown despite detailed examinations. Because of rapid deterioration in the patient's liver function, living donor liver transplantation was performed. She was then diagnosed with hepatic amyloidosis, but died of heart failure due to cardiac amyloidosis 74 days after surgery. Case 2: On the basis of MRCP findings, PSC was suspected in a 49-year-old woman with jaundice. CT showed multiple cystic pulmonary lesions, and hypogammaglobulinemia was also observed (immunoglobulin G, 481 mg/dL). After a biliary plastic stent was placed, liver and lung biopsy confirmed the presence of amyloid deposition. These two cases indicate that it is important to consider hepatic amyloidosis as a differential diagnosis of PSC. The presence of atypical extrahepatic lesions may be useful clues for confirming the diagnosis.

Monitoring systemic amyloidosis using MRI measurements of the extracellular volume fraction

We report the in vivo evaluation, in a murine model, of MRI measurements of the extracellular volume fraction (ECV) for the detection and monitoring of systemic amyloidosis. A new inducible transgenic model was used, with increased production of mouse serum amyloid A protein controlled by oral administration of doxycycline, that causes both the usual hepatic and splenic amyloidosis and also cardiac deposits. ECV was measured in vivo by equilibrium contrast MRI in the heart and liver of 11 amyloidotic and 10 control mice. There was no difference in the cardiac function between groups, but ECV was significantly increased in the heart, mean (standard deviation) 0.20 (0.05) versus 0.14 (0.04), p < 0.005, and liver, 0.27 (0.04) versus 0.15 (0.04), p < 0.0005, of amyloidotic animals and was strongly correlated with the histological amyloid score, myocardium, ρ = 0.67, p < 0.01; liver, ρ = 0.87, p < 0.01. In a further four mice that received human serum amyloid P component (SAP) followed by anti-human SAP antibody, a treatment to eliminate visceral amyloid deposits, ECV in the liver and spleen returned to baseline after therapy (p < 0.01). MRI measurement of ECV is a sensitive marker of amyloid deposits with potential application for early detection and monitoring therapies promoting their clearance.

Endogenous Lipid Pneumonia in an African Grey Parrot (Psittacus erithacus erithacus)

Lipid pneumonia is an unusual, non-infectious, inflammatory lung disease characterized by patchy pneumonic consolidation secondary to accumulation of lipid in macrophages. It can be classified as exogenous or endogenous, depending on whether it is associated with the aspiration of foreign material. Endogenous lipid pneumonia (EnLP) has been reported in many mammal species. In pet birds, EnLP is an incidental and uncommon lesion of unknown pathogenesis. A 35-year-old African grey parrot (Psittacus erithacus erithacus) was presented for depression lasting several months, with marked worsening over the 2 days prior to presentation. The animal died shortly after admission. Necropsy examination showed that the lungs were firm with diffuse grey discolouration. Microscopically, there was EnLP, anthracosis, severe atherosclerosis and hepatic amyloidosis. Although the pathogenesis of EnLP in birds is not clear, it has been associated with concurrent atherosclerosis, hepatic diseases and other lesions of the respiratory tract. This is the first description of EnLP in a bird associated with severe respiratory distress.

Hepatobiliary manifestations in inflammatory bowel disease: The gut, the drugs and the liver

Abnormal liver biochemical tests are present in up to 30% of patients with inflammatory bowel disease (IBD), and therefore become a diagnostic challenge. Liver and biliary tract diseases are common extraintestinal manifestations for both Crohn's disease and ulcerative colitis (UC), and typically do not correlate with intestinal activity. Primary sclerosing cholangitis (PSC) is the most common hepatobiliary manifestation of IBD, and is more prevalent in UC. Approximately 5% of patients with UC develop PSC, with the prevalence reaching up to 90%. Cholangiocarcinoma and colon cancer risks are increased in these patients. Less common disorders include autoimmune hepatitis/PSC overlap syndrome, IgG4-associated cholangiopathy, primary biliary cirrhosis, hepatic amyloidosis, granulomatous hepatitis, cholelithiasis, portal vein thrombosis, liver abscess, and non-alcoholic fatty liver disease. Hepatitis B reactivation during immunosuppressive therapy is a major concern, with screening and vaccination being recommended in serologically negative cases for patients with IBD. Reactivation prophylaxis with entecavir or tenofovir for 6 to 12 mo after the end of immunosuppressive therapy is mandatory in patients showing as hepatitis B surface antigen (HBsAg) positive, independently from viral load. HBsAg negative and anti-HBc positive patients, with or without anti-HBs, should be closely monitored, measuring alanine aminotransferase and hepatitis B virus DNA within 12 mo after the end of therapy, and should be treated if the viral load increases. On the other hand, immunosuppressive therapy does not seem to promote reactivation of hepatitis C, and hepatitis C antiviral treatment does not influence IBD natural history either. Most of the drugs used for IBD treatment may induce hepatotoxicity, although the incidence of serious adverse events is low. Abnormalities in liver biochemical tests associated with aminosalicylates are uncommon and are usually not clinically relevant. Methotrexate-related hepatotoxicity has been described in 14% of patients with IBD, in a dose-dependent manner. Liver biopsy is not routinely recommended. Biologics-related hepatotoxicity is rare, but has been shown most frequently in patients treated with infliximab. Thiopurines have been associated with veno-occlusive disease, regenerative nodular hyperplasia, and liver peliosis. Routine liver biochemical tests are recommended, especially during the first month of treatment. All these conditions should be considered in IBD patients with clinical or biochemical features suggestive of hepatobiliary involvement. Diagnosis and management of these disorders usually involve hepatologists and gastroenterologists due to its complexity.

Clinical characteristics of hepatic amyloidosis in Chinese patients

Clinical characteristics of hepatic amyloidosis in Chinese patients

Hepatic amyloidosis: Morphologic spectrum of histopathological changes in AA and nonAA amyloidosis

In hepatic amyloidosis (HA), the relationships between the pattern and extent of amyloid deposition, morphologic changes, associated diseases and clinical data have not yet been demonstrated. In this study, we sought the correlation between the above mentioned parameters in HA.Liver biopsies of 34 HA were retrospectively analyzed for the type, distribution, and intensity of amyloid deposition and associated morphologic changes. AA and nonAA types were classified on the basis of immunohistochemistry. Follow-up clinical and laboratory findings were reviewed. Twenty-three out of 34 patients (67.6%) had AA, and 11 out of 34 patients (32.4%) had nonAA amyloidosis. The predominant localization pattern in AA amyloidosis was vascular (91.3%), and in nonAA amyloidosis it was mixed with other patterns (72.7%). We confirmed that nonAA amyloid involves the hepatic artery, as well as the portal and central vein, but deposition occurred more frequently in the sinusoidal areas. We detected a portal stromal pattern only in cases of nonAA amyloidosis with a mixed pattern of amyloid deposition.The pattern of amyloid deposition in liver differs between the AA and nonAA type amyloidosis. The distribution of amyloid within the liver is not a reliable method for distinguishing AA from nonAA amyloidosis. However, the histological pattern provides strong clues as to the etiology of the amyloid deposits, and could provide information on the clinical status and prognosis of these patients.

Severe Cholestasis and Portal Hypertension with AV Fistula Formation Secondary to Amyloidosis

Introduction: Systemic amyloidosis encompasses a spectrum of disease characterized by deposition of an abnormal amyloid protein in many organs. Liver involvement in amyloidosis often presents with a cholestatic pattern of liver enzyme elevation although rarely patients may present with portal hypertension or even hepatic failure secondary to amyloid involvement. This is a rare case presentation of acute liver failure secondary to AL (primary) amyloidosis that was initially attributed to congestive heart failure in a patient with known cardiac disease. Case: 86 y/o male with a history of atherosclerotic coronary artery disease presented to Walter Reed National Military Medical Center with a 3 week history of progressive fatigue, lower extremity swelling and new onset elevation of liver associated enzymes in a cholestatic pattern. Physical examination was remarkable for 1+ peripheral edema and hepatomegaly without evidence of ascites or jaundice. Initial laboratory findings revealed an AST of 87 U/L, ALT 29 U/L, alkaline phosphatase of 903 U/L and bilirubin of 2.9 mg/dL. Ultrasound showed diffusely heterogeneous liver parenchyma without ductal dilation or evidence of obstruction. Serologies for autoimmune liver disease, viral hepatitis, and hemochromatosis were negative. Over the course of 1 week, the patient's peripheral edema progressed to anasarca and he became jaundiced. Dedicated CT of the liver showed evidence of an infiltrative process and presence of an Arterio-Venous Fistula in the right hepatic lobe. Paracentesis revealed a SAAG > 1.1 and total protein < 2.0 g/dL. Transjugular liver biopsy was remarkable for elevated trans-sinusoidal pressure of 24 mm Hg consistent with severe portal hypertension. Pathology demonstrated diffuse infiltration of the liver with Congo red staining proteinaceous material consistent with amyloidosis. Serum Kappa light chain levels were notably elevated at 1,102 mg/L. The patient subsequently developed renal failure, with severe hepatic encephalopathy and coagulopathy. His total bilirubin increased to 9.8 mg/dL and the alk phos increased to over 1000 U/L. The patient became anuric and comfort measures were instituted. He passed away on hospital day nine. AL amyloidosis with hepatic involvement carries a poor prognosis with a median survival of 1 year. Presentation with portal hypertension and severe cholestasis occurs in less than 5% of patients and carries a mortality greater than 70%. Spontaneous hepatic AV fistula formation may occur in cirrhosis but has not been reported in the setting of hepatic amyloidosis. This case represents a unique presentation of systemic amyloidosis with hepatic failure as well as an AV fistula.

Hepatic Amyloidosis Presenting as a Large Hepatic Mass

A 43-year-old man was admitted to our department complaining of a large abdominal mass. The patient had become aware of the mass 1 year earlier, but did not seek medical care until it became evident. He denied alcohol and drug abuse, and had no history of chronic hepatitis B or C infection. Physical examination revealed a large, firm, nontender mass that was palpable from the right upper quadrant to the periumbilical area. Laboratory studies revealed high levels of aspartate aminotransferase (122 IU/L), alanine aminotransferase (99 IU/L), total bilirubin (1.4 mg/dL), alkaline phosphatase (2746 IU/L), and γ-glutamyl transpeptidase (709 IU/L).

RE: Imaging Features of Hepato-Splenic Amyloidosis at PET/CT

Regarding the imaging of hepatic amyloidosis with PET/CT, we read with interest the case report by Son and colleagues (1) in the Sep-Oct 2011 issue of the Korean Journal of Radiology. A few points need to be discussed with more detail. First, although the enhanced CT scan of the spleen exhibited a decreased and diffuse parenchymal attenuation, this observation was not sufficiently emphasized within the manuscript. The radiological finding of splenic hypoperfusion has been well described in the literature as a marker of systemic amyloidosis, which presents a useful clue when clinical findings fail to determine the proper diagnosis (2). In the case reported by Son and colleagues, if the radiologist had immediately suggested the correct diagnostic hypothesis, the patient would have been directed to have the the appropriate

Primary hepatic amyloidosis: A case report

Primary hepatic amyloidosis: A case report

Too Much Protein Has Made Me Pale

Purpose: Hepatic Amyloidosis is a type of primary amyloidosis, which is characterized by extracellular deposition of insoluble fibrils derived from immunoglobulin light chains. Primary systemic or AL (amyloid light chain) amyloidosis is the most common cause of amyloidosis in the Western world, but remains a rare disorder with an estimated annual incidence of about 3000 cases in the United States. Case Report: A 45 year old man with a past medical history of anemia presents with a one-month history of generalized weakness, 18 lbs wt loss and malaise. Endoscopy, colonoscopy and video capsule study was performed onemonth prior, which was unremarkable. Significant findings on physical exam included a liver span of approximately 10 cm below the costal margin and pale conjunctiva. Laboratory results were significant for alkaline phosphatase-783, alanine aminotransferase-59, aspartate aminotransferase-72 and total bilirubin-1.8. Computed tomography of the abdomen and pelvis revealed an enlarged heterogeneous appearing liver. Biopsy of the liver was consistent with amyloidlight chain type (Figure 1). Patient was subsequently diagnosed with Multiple Myeloma. Conclusion: Chronic anemia is a common indication for outpatient endoscopic evaluation. However, in cases where findings are inconclusive, other potential sources should be considered. In our case, the physical exam was pertinent for hepatomegaly, which prompted us to consider infiltrative diseases of the liver. The Liver biopsy is the gold standard in diagnosing hepatic amyloid. However, new non-invasive tools, such as the fibroscan may aid in the diagnosis hepatic amyloid. In this case, AL amyloidosis although rare, led to the diagnosis of multiple myeloma. Furthermore, in the era of stem cell transplant and chemotherapy, hepatic amyloid is treatable and can potentially be cured.Figure: No Caption available.

Typhlocolitis associated with spirochaetes in duck flocks

The aetiology of increased mortality observed in two breeder duck flocks (Flock A consisting of 3500 laying ducks and Flock B comprising 4300 laying ducks) during the first egg-laying season was studied. In Flocks A and B, 773 ducks and 715 ducks (18.4% and 16.6%) died within a 24-week and a 20-week period, respectively. Death was preceded by clinical signs including movement difficulties, lack of appetite and depression lasting for 1 to 2 days. Diarrhoea was not observed. On gross pathological examination, the ducks were found to have haemorrhagic to fibrinonecrotic typhlocolitis, renal degeneration accompanied by fibrosis and mineralization, hepatic and splenic amyloidosis, and swelling of some of the metatarsal and phalangeal joints. Histopathological and immunohistochemical examination consistently demonstrated spirochaetes in the mucous membrane of the affected large intestine. On the basis of their cultural and biochemical properties and polymerase chain reaction sequencing analysis, four out of seven spirochaete strains isolated from the ducks (Flock A) by culture on special media under anaerobic conditions were identified as Brachyspira hyodysenteriae, and five out of eight strains (Flock B) were identified as Brachyspira pilosicoli. This is the first report on the isolation of B. hyodysenteriae and B. pilosicoli from laying ducks affected by fibrinonecrotic typhlocolitis.

Non-invasive detection of hepatic amyloidosis: FibroScan, a new tool

Introduction. FibroScan, a non-invasive tool for measuring liver stiffness (LS), is not specific to liver fibrosis. Other extra-hepatic conditions may modify the LS value.Objectives. Our aim was to examine whether amyloid deposition in the liver may modify LS.Methods. LS was measured prospectively in 41 patients with systemic AL amyloidosis (AL) in the French AL Reference Center, comprising: 5 patients with liver involvement (LI) and no cardiac involvement (CI), 11 with CI and no LI, 12 with both LI and CI and 13 with neither (2005 consensus criteria); 26 negative controls, 50 patients infected with Hepatitis C virus (HCV)-infected and 18 AL-free patients with right-sided heart disease (‘cardiac controls’) were also examined.Results. Median LS was significantly higher in patients with AL with liver involvement [27.4 (10.3–75) kPa] than in negative controls [4.8 (2.8–11.9) kPa] (p < 0.0001), and patients infected with HCV [(6.8 (2.9–69.1) kPa] (p = 0.001), and tended to be higher than in the ‘cardiac controls’ [11 (4.1–75) kPa] (p = 0.08). A cut-off value of 17.3 kPa, prioritising specificity, is proposed for routine diagnosis of significant AL liver infiltration.Conclusion. LS > 17.3 kPa is suggestive of AL hepatic disease in patients with non-fibrotic liver changes, and may have diagnostic value in patients with known AL.

18F-FDG PET/CT in Primary AL Hepatic Amyloidosis Associated with Multiple Myeloma

We report here on a rare case of primary AL hepatic amyloidosis associated with multiple myeloma in a 64-year-old woman. The patient was referred for evaluating her progressive jaundice and right upper quadrant pain. 18F-fluorodeoxyglucose (18F-FDG) positron emission tomography (PET)/computed tomography (CT) showed diffusely and markedly increased 18F-FDG uptake in the liver. Although there have been several case studies showing positive 18F-FDG uptake in pulmonary amyloidosis, to the best of our knowledge, the 18F-FDG PET/CT findings of hepatic amyloidosis or primary hepatic amyloidosis associated with multiple myeloma have not been reported previously.