LIGHT CHAIN DEPOSITION DISEASE OF THE LIVER ASSOCIATED WITH AL-TYPE AMYLOIDOSIS AND SEVERE CHOLESTASIS - A CASE-REPORT AND LITERATURE-REVIEW

被引:37
作者
FAA, G [1 ]
VANEYKEN, P [1 ]
DEVOS, R [1 ]
FEVERY, J [1 ]
VANDAMME, B [1 ]
DEGROOTE, J [1 ]
DESMET, VJ [1 ]
机构
[1] CATHOLIC UNIV LEUVEN, UNIV ZIEKENHUIS GASTHUISBERG, INWENDIGE GENEESKUNDE HEPATOL, B-3030 HEVERLEE, BELGIUM
关键词
D O I
10.1016/0168-8278(91)90913-V
中图分类号
R57 [消化系及腹部疾病];
学科分类号
摘要
A 67-year-old many with a 3-month history of jaundice presented with hepatomegaly. Laboratory studies revealed abnormal liver tests with raised bilirubin. Renal function was normal. Endoscopic retrograde cholangiopancreatography revealed normal extrahepatic bile ducts. Liver biopsy showed severe bilirubinostasis and a typical bile infarct. Laminar and globular deposits of PAS-positive diastase-resistant non-congophilic material were observed in the sinusoidal walls. In addition, congophilic material was detected in the portal tracts. Immunohistochemistry revealed the present of lambda-light chain deposits both in the sinusoids and in the portal tracts. Collagens type I and IV and fibronectin appeared markedly increased in the perisinusoidal space. On electron microscopy, the deposited material in the Disse spaces was mainly composed of fibrils indistinguishable from amyloid, admixed with small amounts of granular electron-dense material. The similarities of light chain deposition disease and AL amyloidosis are discussed.
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页码:75 / 82
页数:8
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