Severe recurrent intrahepatic cholestasis in systemic AL amyloidosis without evident liver involvement. Unexplained hepatic toxicity or a case of misdiagnosed liver amyloidosis?

摘要

We report on a 50-year old female admitted for acute abdominal pain, nephrotic proteinuria, and rapidly progressive renal failure with mild BJ proteinuria, negative serum- immunofixation (IFX), and a mild increase of X free light chains (FLC). A bone marrow biopsy and a fat tissue aspirate showed multiple myeloma with AL amyloidosis. The patient started therapy with bortezomib (B) 1.3 mg/m~2 plus dexamethasone 40 mg on days 1, 4, 8, and 11 and because of end stage renal disease (ESRD) began dialysis therapy. Ten days later, she complained of a new episode of abdominal pain with jaundice that recovered completely after four cycles of B. A CT scan and NMR ruled out all secondary causes of cholangitis including neoplasms. Acute intrahepatic cholestasis due to amyloid deposition was then hypothesized. After 3 months, a new episode occurred and this time she underwent surgery for an intestinal volvolus. Intestinal and liver biopsies, however, showed no evidence of amyloid deposits.