title: [Severe recurrent intrahepatic cholestasis in systemic AL amyloidosis without obvious liver involvement: unexplained hepatic toxicity or a case of misdiagnosed liver amyloidosis?].
abstract: We report the case of a 50-year-old woman who was admitted to the hospital for acute abdominal pain with nephrotic proteinuria, rapidly progressive renal failure, and moderate anemia. Laboratory tests showed mild Bence Jones (λ) proteinuria with negative serum immunofixation and a mild increase in λ free light chains. A bone marrow biopsy and a fat tissue aspirate showed multiple myeloma and amyloidosis.         Because of the end-stage renal disease, the patient began regular dialysis treatment and was started on bortezomib 1.3 mg/m2 plus dexamethasone 40 mg on days 1, 4, 8 and 11 of 21-day cycles. Ten days later she complained of a new episode of abdominal pain with jaundice. A CT scan and an MRI scan ruled out all secondary causes of cholangitis including cancer. Acute intrahepatic cholestasis due to amyloid deposition was then hypothesized. After 4 well tolerated cycles of bortezomib and dexamethasone, blood tests showed a complete hematological response with full reversal of cholestasis. After three months, a new episode of abdominal pain occurred and this time the patient was operated on and found to have an intestinal volvulus. Because of the jaundice, a transjugular liver biopsy was performed showing no evidence of amyloid deposits. Two months later the patient died of septic shock. Although no autopsy was performed and the ultimate cause of the cholestasis could not be ascertained, amyloidosis remains the major culprit in this unfortunate case.
authors.affiliation: Unita' Operativa di Nefrologia dei Trapianti, A.O.U. Careggi, Firenze, Italy. paudicenunzia@libero.it
authors.forename: Nunzia
authors.initials: N
authors.lastname: Paudice
authors.forename: Silvia
authors.initials: S
authors.lastname: Farsetti
authors.forename: Leonardo
authors.initials: L
authors.lastname: Caroti
authors.forename: Sandro
authors.initials: S
authors.lastname: Bandini
authors.forename: Gabriele
authors.initials: G
authors.lastname: Ciuti
authors.forename: Alessio
authors.initials: A
authors.lastname: Tempestini
authors.forename: Federico
authors.initials: F
authors.lastname: Perfetto
authors.forename: Simone
authors.initials: S
authors.lastname: Galli
authors.forename: Letizia
authors.initials: L
authors.lastname: Giabbani
authors.forename: Anna Lucia
authors.initials: AL
authors.lastname: Caldini
authors.forename: Franco
authors.initials: F
authors.lastname: Bergesio
mesh_terms: D000686:Amyloidosis / Q000150:complications* / Q000175:diagnosis*; D056486:Chemical and Drug Induced Liver Injury / Q000175:diagnosis*; D002780:Cholestasis, Intrahepatic / Q000209:etiology*; D003951:Diagnostic Errors; D005260:Female; D006801:Humans; D008107:Liver Diseases / Q000175:diagnosis*; D008875:Middle Aged; D012008:Recurrence; D012720:Severity of Illness Index