Severe intrahepatic cholestasis, erythrocytosis and hypoglycemia: unusual presenting features of systemic AL amyloidosis.

Abstract:

:We report the case of a 68-year-old African woman who presented with jaundice, hepatomegaly and anasarca. Clinical investigation disclosed severe intrahepatic cholestasis, nephrotic syndrome, erythrocytosis and hypoglycemia. Diagnosis of systemic AL amyloidosis was established by percutaneous liver biopsy. Bone marrow biopsy showed 32% of myeloma cells. The patient started treatment with melphalan and prednisolone, but liver function deteriorated and she died in hepatic failure complicated by septic shock three weeks after the diagnosis. We present possible explanations for the unusual clinical and laboratory findings, which required a multidisciplinary approach and posed challenging problems in differential diagnosis and management.

journal_name

Scand J Gastroenterol

authors

Ferreira S,Baldaia C,Fatela N,Ramalho F,Costa MA,Brito D,Esteves G,Monteiro E

doi

10.1080/00365520701679298

subject

Has Abstract

pub_date

2008-03-01 00:00:00

pages

375-9

issue

3

eissn

0036-5521

issn

1502-7708

journal_volume

43

pub_type

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