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• W1964996825 abstract "Question: A 52-year-old man was referred to our clinic for evaluation of cirrhosis. The patient had noticed increasing abdominal girth for 3 months. He drank alcohol almost daily since the age of 16, up to 8 beers per day. He stopped drinking 1 month prior. On physical examination, the patient appeared chronically ill with scleral icterus. His abdomen was tense. The liver was enlarged 5–8 cm below the costal margin. Laboratory data revealed aspartate aminotransferase, 129 U/L; alanine aminotransferase, 77 U/L; alkaline phosphatase (ALP), 846 U/L; total bilirubin, 3.1 mg/dL; platelets, 671 k/μL; creatinine, 0.7 mg/dL; and International Normalized Ratio, 1.1. Computed tomography of the abdomen revealed a large amount of ascites, hepatomegaly, decreased attenuation of the liver suggesting fatty liver, and a recanalized umbilical vein. These changes were suspicious of portal hypertension attributed to alcoholic cirrhosis.Owing to a persistently elevated alkaline phosphatase on repeat testing (1164 U/L), out of proportion to what would be expected in alcoholic cirrhosis, a liver biopsy was obtained. The hematoxylin and eosin stain of the liver biopsy revealed replacement of the normal lobular architecture by abundant deposition of amorphous eosinophilic material in a sinusoidal and nodular pattern (Figure A). An upper endoscopy, done for screening of varices, showed gastric and small bowel biopsies with positive Congo red stains (Figure B) with green birefringence under polarized light (Figure C).What is your diagnosis?See the Gastroenterology web site (www.gastrojournal.org) for more information on submitting your favorite image to Clinical Challenges and Images in GI.Answer to the Clinical Challenges and Images in GI Question: Image 3: Primary Hepatic Amyloidosis Causing Portal HypertensionThe thioflavin S immunofluorescent stain on the liver biopsy for amyloid was strongly positive. Transthyretin and amyloid A immunostains were negative in the amyloid deposits, making primary amyloidosis the most likely diagnosis. Further testing revealed an immunoglobulin lambda monoclonal protein associated with amyloid infiltrating the liver and the bone marrow. He was treated with 6 cycles of bortezomib and dexamethasone with complete remission of his immunoglobulin lambda monoclonal protein. His ALP was 313 U/L and total bilirubin 0.4 mg/dL at the end of treatment. One year later, he presented with confusion and recurrent ascites. His ALP increased to 2319 U/L and total bilirubin was 5.6 mg/dL. Gastric biopsies confirmed the recurrence of his amyloid. Owing to rapidly progressive liver failure, he died before further treatment could be initiated.Primary systemic amyloidosis most commonly affects the heart, kidney, and peripheral nerves. The liver has also been identified as a common site.1Buck F.S. Koss M.N. Hepatic amyloidosis: morphologic differences between systemic AL and AA type.Hum Pathol. 1991; 22: 904-907Crossref PubMed Scopus (83) Google Scholar A study of 98 patients with primary systemic amyloidosis and biopsy-proven liver involvement found that patients presented most commonly with nonspecific symptoms such as involuntary weight loss, edema, or abdominal pain.2Park M.A. Mueller P.S. Kyle R.A. et al.Primary (AL) hepatic amyloidosis: clinical features and natural history in 98 patients.Medicine. 2003; 82: 291-298Crossref PubMed Scopus (173) Google Scholar Alkaline phosphatase is often elevated in hepatic amyloid. On the hematoxylin and eosin stain, the amyloid appears as a pink, amorphous, waxy substance and is most often encountered in a perisinusoidal distribution. The fibrils bind Congo red and, under polarized light, there is a green birefringence. The fibrils also bind thioflavin T and S, which produces an intense yellow-green fluorescence. Median survival for patients is poor, at about 8.5 months.2Park M.A. Mueller P.S. Kyle R.A. et al.Primary (AL) hepatic amyloidosis: clinical features and natural history in 98 patients.Medicine. 2003; 82: 291-298Crossref PubMed Scopus (173) Google Scholar Factors contributing to worse survival include an elevated bilirubin, congestive heart failure, and a platelet count >500 × 109/L. Despite the poor prognosis of primary hepatic amyloidosis, some patients may benefit from treatment such as systemic chemotherapy. Question: A 52-year-old man was referred to our clinic for evaluation of cirrhosis. The patient had noticed increasing abdominal girth for 3 months. He drank alcohol almost daily since the age of 16, up to 8 beers per day. He stopped drinking 1 month prior. On physical examination, the patient appeared chronically ill with scleral icterus. His abdomen was tense. The liver was enlarged 5–8 cm below the costal margin. Laboratory data revealed aspartate aminotransferase, 129 U/L; alanine aminotransferase, 77 U/L; alkaline phosphatase (ALP), 846 U/L; total bilirubin, 3.1 mg/dL; platelets, 671 k/μL; creatinine, 0.7 mg/dL; and International Normalized Ratio, 1.1. Computed tomography of the abdomen revealed a large amount of ascites, hepatomegaly, decreased attenuation of the liver suggesting fatty liver, and a recanalized umbilical vein. These changes were suspicious of portal hypertension attributed to alcoholic cirrhosis. Owing to a persistently elevated alkaline phosphatase on repeat testing (1164 U/L), out of proportion to what would be expected in alcoholic cirrhosis, a liver biopsy was obtained. The hematoxylin and eosin stain of the liver biopsy revealed replacement of the normal lobular architecture by abundant deposition of amorphous eosinophilic material in a sinusoidal and nodular pattern (Figure A). An upper endoscopy, done for screening of varices, showed gastric and small bowel biopsies with positive Congo red stains (Figure B) with green birefringence under polarized light (Figure C). What is your diagnosis? See the Gastroenterology web site (www.gastrojournal.org) for more information on submitting your favorite image to Clinical Challenges and Images in GI. Answer to the Clinical Challenges and Images in GI Question: Image 3: Primary Hepatic Amyloidosis Causing Portal HypertensionThe thioflavin S immunofluorescent stain on the liver biopsy for amyloid was strongly positive. Transthyretin and amyloid A immunostains were negative in the amyloid deposits, making primary amyloidosis the most likely diagnosis. Further testing revealed an immunoglobulin lambda monoclonal protein associated with amyloid infiltrating the liver and the bone marrow. He was treated with 6 cycles of bortezomib and dexamethasone with complete remission of his immunoglobulin lambda monoclonal protein. His ALP was 313 U/L and total bilirubin 0.4 mg/dL at the end of treatment. One year later, he presented with confusion and recurrent ascites. His ALP increased to 2319 U/L and total bilirubin was 5.6 mg/dL. Gastric biopsies confirmed the recurrence of his amyloid. Owing to rapidly progressive liver failure, he died before further treatment could be initiated.Primary systemic amyloidosis most commonly affects the heart, kidney, and peripheral nerves. The liver has also been identified as a common site.1Buck F.S. Koss M.N. Hepatic amyloidosis: morphologic differences between systemic AL and AA type.Hum Pathol. 1991; 22: 904-907Crossref PubMed Scopus (83) Google Scholar A study of 98 patients with primary systemic amyloidosis and biopsy-proven liver involvement found that patients presented most commonly with nonspecific symptoms such as involuntary weight loss, edema, or abdominal pain.2Park M.A. Mueller P.S. Kyle R.A. et al.Primary (AL) hepatic amyloidosis: clinical features and natural history in 98 patients.Medicine. 2003; 82: 291-298Crossref PubMed Scopus (173) Google Scholar Alkaline phosphatase is often elevated in hepatic amyloid. On the hematoxylin and eosin stain, the amyloid appears as a pink, amorphous, waxy substance and is most often encountered in a perisinusoidal distribution. The fibrils bind Congo red and, under polarized light, there is a green birefringence. The fibrils also bind thioflavin T and S, which produces an intense yellow-green fluorescence. Median survival for patients is poor, at about 8.5 months.2Park M.A. Mueller P.S. Kyle R.A. et al.Primary (AL) hepatic amyloidosis: clinical features and natural history in 98 patients.Medicine. 2003; 82: 291-298Crossref PubMed Scopus (173) Google Scholar Factors contributing to worse survival include an elevated bilirubin, congestive heart failure, and a platelet count >500 × 109/L. Despite the poor prognosis of primary hepatic amyloidosis, some patients may benefit from treatment such as systemic chemotherapy. The thioflavin S immunofluorescent stain on the liver biopsy for amyloid was strongly positive. Transthyretin and amyloid A immunostains were negative in the amyloid deposits, making primary amyloidosis the most likely diagnosis. Further testing revealed an immunoglobulin lambda monoclonal protein associated with amyloid infiltrating the liver and the bone marrow. He was treated with 6 cycles of bortezomib and dexamethasone with complete remission of his immunoglobulin lambda monoclonal protein. His ALP was 313 U/L and total bilirubin 0.4 mg/dL at the end of treatment. One year later, he presented with confusion and recurrent ascites. His ALP increased to 2319 U/L and total bilirubin was 5.6 mg/dL. Gastric biopsies confirmed the recurrence of his amyloid. Owing to rapidly progressive liver failure, he died before further treatment could be initiated. Primary systemic amyloidosis most commonly affects the heart, kidney, and peripheral nerves. The liver has also been identified as a common site.1Buck F.S. Koss M.N. Hepatic amyloidosis: morphologic differences between systemic AL and AA type.Hum Pathol. 1991; 22: 904-907Crossref PubMed Scopus (83) Google Scholar A study of 98 patients with primary systemic amyloidosis and biopsy-proven liver involvement found that patients presented most commonly with nonspecific symptoms such as involuntary weight loss, edema, or abdominal pain.2Park M.A. Mueller P.S. Kyle R.A. et al.Primary (AL) hepatic amyloidosis: clinical features and natural history in 98 patients.Medicine. 2003; 82: 291-298Crossref PubMed Scopus (173) Google Scholar Alkaline phosphatase is often elevated in hepatic amyloid. On the hematoxylin and eosin stain, the amyloid appears as a pink, amorphous, waxy substance and is most often encountered in a perisinusoidal distribution. The fibrils bind Congo red and, under polarized light, there is a green birefringence. The fibrils also bind thioflavin T and S, which produces an intense yellow-green fluorescence. Median survival for patients is poor, at about 8.5 months.2Park M.A. Mueller P.S. Kyle R.A. et al.Primary (AL) hepatic amyloidosis: clinical features and natural history in 98 patients.Medicine. 2003; 82: 291-298Crossref PubMed Scopus (173) Google Scholar Factors contributing to worse survival include an elevated bilirubin, congestive heart failure, and a platelet count >500 × 109/L. Despite the poor prognosis of primary hepatic amyloidosis, some patients may benefit from treatment such as systemic chemotherapy." @default.
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• W1964996825 title "Elevated Alkaline Phosphatase in a Man With Ascites" @default.
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