Systemic amyloidosis manifesting as a rare cause of hepatic failure.
In 1854, the term "amyloid" was first used in the description of a liver specimen at autopsy by Virchow. The kidneys and heart are the most commonly involved organs in amyloidosis; liver and gastrointestinal tract involvement is less common, and the symptoms are usually mild. Here, we report the case of a 57-year-old male patient who presented with oral hemorrhagic bullae, thrombocytopenia and jaundice. Disseminated intravascular coagulation profile was positive. Abdominal sonography showed ascites, and abdominal computed tomography disclosed heterogeneous enhancement of the liver, with focal low attenuation regions and splenomegaly with poor contrast enhancement. Liver decompensation was highly suspected. Diagnostic laparoscopy with liver biopsy and colonoscopic biopsy from the rectum were subsequently performed. Typical apple-green birefringence was demonstrated on polarized light microscopy by Congo red staining. Systemic amyloidosis was diagnosed and colchicine prescribed. However, liver function deteriorated and intermittent gastrointestinal bleeding was found during the patient's hospitalization. The patient died due to uncorrectable coagulopathy and massive gastrointestinal bleeding. The final diagnosis was idiopathic amyloidosis with hepatic failure. Although amyloidosis rarely presents with hepatic failure, it should be considered in patients with signs of liver decompensation. Clinicians should be aware of this rare but potentially lethal presentation and arrange appropriate treatment promptly.
Hung
Hung-Hsu
HH
Division of Gastroenterology, Taipei Veterans General Hospital, Taipei, Taiwan, R.O.C.
Huang
De-Feng
DF
Tzeng
Cheng-Hwai
CH
Su
Cheng-Hsi
CH
Su
Tung-Ping
TP
Chen
Hung-Chieh
HC
Tsay
Shyh-Haw
SH
Lin
Han-Chieh
HC
Wu
Jaw-Ching
JC
Lee
Shou-Dong
SD
Su
Chien-Wei
CW
D000686:Amyloidosis / Q000150:complications*; D006801:Humans; D017093:Liver Failure / Q000209:etiology*; D008297:Male; D008875:Middle Aged