Hepatic amyloidosis: a cause of rapidly progressive jaundice

Abstract

DesCripTionAn 83-year-old man presented with an acute history of weight loss and jaundice. He had a history of type 2 diabetes mellitus and hypertension. He consumed 30 units of alcohol per week.The patient was cachectic and jaundiced with non-tender hepatomegaly and no evidence of chronic liver disease. There was evidence of hypoalbuminaemia (albumin 25 g/L, reference 34-51 g/L), hyperbilirubinaemia (bilirubin 188 μmol/L, reference <22 μmol/L) and a raised alkaline phosphatase (629 IU/L, reference 35-105 IU/L). Full blood count, coagulation tests and the remaining liver function tests were normal. An estimated Glomerular filtration rate (eGFR) was 71 mL/ min/1.73 m 2 . Autoantibodies and immunoglobulins were normal. Hepatitis viral serology was negative. Serum light chain measurements revealed kappa chain concentration of 13.3 (reference 3.3-19.4 mg/L) and lambda chain concentration of 28.5 (reference 5.7-26.6 mg/L) with a ratio of 0.47 (reference 0.26-1.75). A CT abdomen revealed hepatomegaly and ascites.

Hepatic amyloidosis: a prevalence study and clinical characterization of a rare and severe disease

“…There are cases of hepatic amyloidosis that present as rapidly progressive liver failure with hyperbilirubinemia that usually demonstrates an unfavorable evolution with multi-organ dysfunction and short-term survival (15)(16)(17). This is also reflected in our series, where one patient presented with rapidly progressive liver failure with marked conjugated hyperbilirubinemia and survived only three weeks after diagnosis.…”

Along for the Ride: Intrahepatic Cholangiocarcinoma with Concomitant LECT2 Amyloidosis

“…Patients with systemic amyloidosis usually have liver involvement. In the clinical setting, patients with hepatic amyloidosis may present with hepatomegaly, ascites, cholestasis, jaundice, or portal hypertension [11][12][13][14]. Liver biopsies show sinusoidal and intravascular amyloid deposition for both AL and AA amyloidosis; thus, the deposition pattern cannot distinguish AL from AA amyloid [8].…”

Spontaneous hepatic rupture due to primary amyloidosis

“…Although rare, amyloidosis must be considered when other causes for presentation similar to our patient are ruled out. A number of cases have been reported describing liver/splenic rupture from amyloidosis with mostly poor clinical outcomes 1–7…”