Light chain deposition disease of the liver associated with AL-type amyloidosis and severe cholestasis.
A 67-year-old man 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 presence 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.
Faa
G
G
Laboratorium voor Histo- en Cytochemie, Dienst Pathologische Ontleedkunde II, U.Z. St. Rafael, Leuven, Belgium.
Van Eyken
P
P
De Vos
R
R
Fevery
J
J
Van Damme
B
B
De Groote
J
J
Desmet
V J
VJ
D000368:Aged; D000686:Amyloidosis / Q000150:complications* / Q000378:metabolism / Q000473:pathology; D001663:Bilirubin / Q000378:metabolism; D002779:Cholestasis / Q000150:complications* / Q000378:metabolism / Q000473:pathology; D003094:Collagen / Q000378:metabolism; D005353:Fibronectins / Q000378:metabolism; D006801:Humans; D007147:Immunoglobulin Light Chains / Q000378:metabolism*; D007075:Immunoglobulin M / Q000378:metabolism; D007145:Immunoglobulin kappa-Chains / Q000378:metabolism; D007150:Immunohistochemistry; D007797:Laminin / Q000378:metabolism; D008099:Liver / Q000378:metabolism / Q000473:pathology / Q000648:ultrastructure; D008107:Liver Diseases / Q000150:complications* / Q000378:metabolism / Q000473:pathology; D008297:Male; D008854:Microscopy, Electron
D005353:Fibronectins; D007147:Immunoglobulin Light Chains; D007075:Immunoglobulin M; D007145:Immunoglobulin kappa-Chains; D007797:Laminin; D003094:Collagen; D001663:Bilirubin