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Query: UMLS:C0019209 (hepatomegaly)
 5,798 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Amyloidosis is classified according to the distribution pattern of amyloid deposition sites and associated diseases. Hepatic amyloidosis is not infrequent, although rarely causes clinical liver disease. We report two cases of amyloidosis diagnosed by liver biopsy. One presented with symptoms related almost to the liver disease, such as jaundice, hepatomegaly and indigestion. Echocardiogram revealed hypertrophic cardiomyopathy, suggesting cardiac involvement of the amyloidosis. The patient died of hepatic failure. The other case was found in a patient with an end stage renal disease. Features of congestive heart failure in this case may reflect cardiac involvement. The pattern of hepatic amyloid deposition in both of these cases was diffuse perisinusoidal. The predominant intralobular deposition suggests that these are amyloidosis of the secondary type.
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PMID:Hepatic amyloidosis--two cases report. 326 63

Primary systemic amyloidosis (AL) is an uncommon disease characterized by the extracellular deposition of a protein with a beta-fibrillar structure, consisting of monoclonal immunoglobulin light chains, lambda or kappa (ratio of lambda to kappa, 3:1). In systemic amyloidosis liver involvement is frequent but it rarely has clinical importance. The massive and localized liver deposition of amyloid, characterized by marked hepatomegaly and portal hypertension without hepato-cellular failure and by a severe prognosis, without systemic involvement, is less frequent. The authors describe an unusual case of primary hepatic amyloidosis with giant hepatomegaly, intrahepatic cholestasis, portal hypertension and splenomegaly, occurred in an elderly patient.
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PMID:Giant hepatomegaly and portal hypertension in an elderly patient with primary liver amyloidosis: an uncommon clinical occurrence. 1140 89

Hepatic amyloidosis complicated with Castleman's disease is quite rare. A 48-year-old woman was referred to our hospital with general fatigue, low-grade fever, anemia, thrombocythemia, and liver dysfunction. Physical examination revealed anemia and hepatomegaly and abdominal computed tomography showed marked hepatomegaly and right upper abdominal masses. Technetium-99m pyrophosphate (99mTc-PYP) scintigraphy revealed the diffuse abnormal uptake of the enlarged liver, suggesting amyloid deposition. Liver biopsy showed destruction of the liver structure and the massive deposition of AA type amyloid protein. Surgical resection was performed on the abdominal masses. Histological examination of the masses showed Castleman's disease (plasma cell type). After resection, her fever resolved and the liver size gradually decreased to within the normal range. This case shows that surgical resection of the main lesion is effective for hepatomegaly due to AA type amyloidosis associated with Castleman's disease.
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PMID:Marked hepatomegaly due to AA type amyloidosis in a case with Castleman's disease. 1686 6