Intrahepatische Cholestase als Leitsymptom einer primären Amyloidose

Abstract

In a 37-year-old female patient with marked signs of cholestasis and excessive hyperbilirubinaemia without any recognisable cause, the unexpected diagnosis of massive amyloidosis of the peri-collagenous and perireticular type was made after hepatic needle biopsy. There were also amyloid deposits in the rectal mucosa and in the bone marrow. Fifteen months after the onset of disease symptoms the patient died from the consequences of spontaneous intrahepatic haemorrhage. At the post-mortem investigation generalised amyloidosis with massive involvement of nearly all organs was found. Primary amyloidosis should be considered in the differential diagnosis of any aetiologically unexplained cholestasis.

Fatal Spontaneous Liver Rupture in a Renal Transplant Patient with Amyloidosis

“…It has been described in 6 cases associated with amyloidosis [1][2][3][4][5], but in a computer ized search using MEDLINE we did not find a single case in kidney transplant patients. Spontaneous liver rupture in amyloidosis has occasionally been related to minimal trauma [1] or laparoscopic procedures [4], It is usually associated with severe intra-ab dominal hemorrhage, that causes fulminant death, although a case has been described with fatal intrahepatic hemorrhage without rupture of the liver [6]. Many factors could be involved in bleeding and rupture: (1) re duced hepatic tissue pliability secondary to amyloid infiltration; (2) increased vascular fragility as a result of infiltration of blood vessel walls [7]; (3) coagulation abnormali ties, such as acquired factor X deficiency [8], increased fibrinolysis [9] or disseminated in travascular coagulation.…”