AL amyloidosis

A plasma cell disorder characterized by the aggregation and deposition of insoluble amyloid fibrils derived from misfolding of monoclonal immunoglobulin light chains usually produced by a plasma cell tumor. It usually presents as primary systemic amyloidosis (PSA) with multiple organ involvement and less frequently as primary localized amyloidosis (PLA) restricted to a single organ.

Cholestasis

Impairment of bile flow due to obstruction in bile ducts.


Total: 7

                       


(per page)
PMID (PMCID)
26965175
 MALE Adult
Acute liver failure due to primary amyloidosis in a nephrotic syndrome: a swiftly progressive course.
Cardoso BA, Leal R, Sa H, Campos M.
BMJ Case Rep. 2016;2016:.
We report a case of a 39-year-old man admitted to our nephrology department with an unusual presentation of primary amyloidosis with nephrotic syndrome and acute liver failure, complicated by obstructive cholestasis resulting in death 28197months after diagnosis.
17592572
 FEMALE Middle Aged
Primary amyloidosis presenting with cholestasis and hyperkinetic portal hypertension.
Culafic D, Perisic M, Boricic I, Culafic-Vojinovic V, Vukcevic M.
J Gastrointestin Liver Dis. 2007;16(2):201-4.
Primary amyloidosis presenting with cholestasis and hyperkinetic portal hypertension.
12472208
 MALE
Cholestasis and renal failure in a patient with secondary amyloidosis.
Unsal C, Paydas S, Gonlusen G.
Ren Fail. 2002;24(6):863-6.
There are a few cases that secondary amyloidosis presented with hepatic involvement and cholestasis which seems to be limited to primary amyloidosis.
9684440
 MALE
A case of intrahepatic cholestasis due to amyloidosis.
McDonald P, Usbourne C, Playfer JR.
Int J Clin Pract. 1998;52(3):201-2.
We report a case of cholestasis and hepatic failure in an 85-year-old man due to primary amyloidosis.
9820409
 MALE Middle Aged
Primary amyloidosis with spontaneous splenic rupture, cholestasis, and liver failure treated with emergency liver transplantation.
Sandberg-Gertzen H, Ericzon BG, Blomberg B.
Am J Gastroenterol. 1998;93(11):2254-6.
Primary amyloidosis with spontaneous splenic rupture, cholestasis, and liver failure treated with emergency liver transplantation.
3115870
 MALE
Cholestasis and liver failure with lambda-AL amyloidosis.
Konikoff F, Mor C, Stern S, Shaklai M, Halevy J, Theodor E.
Gut. 1987;28(7):903-6.
Cholestasis and liver failure with lambda-AL amyloidosis.
1001194
 FEMALE Adult
[Primary amyloidosis presenting as intrahepatic cholestasis (author's transl)].
Rollinghoff W, Braun HJ, Schad FJ.
Dtsch Med Wochenschr. 1976;101(50):1838-41.
Primary amyloidosis should be considered in the differential diagnosis of any aetiologically unexplained cholestasis.