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Annales de Biologie Clinique. Volume 63, Number 5, 519-23, Septembre-Octobre 2005, pratique quotidienne

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Author(s) : D Swortfiguer, Y Bacq, F Schellenberg, A de Muret, L Benboubker, E Piver, J-C Pagès

Summary : 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.

Keywords : non-icteric cholestatsis, nephritic syndrome, AL amyloidosis