[An anicteric cholectasis and nephrotic syndrome: AL amyloïdosis in a 51 years-old man].
Amyloidosis is a multiple-organ disease for which the diagnosis is often confusing and thereby delayed. Here, we present an archetypal case illustrating such difficulties. A 51 years-old man presented a mixed dyslipemia in November 2002, in June 2004 he has finally been diagnosed with a primary AL-amyloidosis. Within these two years, the arising of a non-icteric cholestasis and a nephrotic syndrome have triggered the search for a disease related to a multiple-organ protein deposition. Confirmation of the AL-amyloidosis was obtained through an histological examination, including direct immuno-fluorescence. Amyloidosis is a life threatening disease that need to be diagnosed at an early stage, in order to maximise the therapeutic expectations. The average survival after the diagnosis of AL-amyloidosis is 5% at 10 years. Often, treatments are initiated late in the course of the disease, at a time when organ lesion are constituted, severely affecting the prognosis.
Swortfiguer
D
D
Service de biochimie et biologie moléculaire, CHRU Tours.
Bacq
Y
Y
Schellenberg
F
F
de Muret
A
A
Benboubker
L
L
Piver
E
E
Pagès
J-C
JC
D000686:Amyloidosis / Q000150:complications* / Q000473:pathology; D001706:Biopsy; D002780:Cholestasis, Intrahepatic / Q000150:complications* / Q000473:pathology; D003937:Diagnosis, Differential; D006801:Humans; D008099:Liver / Q000473:pathology; D008297:Male; D008875:Middle Aged; D009404:Nephrotic Syndrome / Q000150:complications* / Q000473:pathology