Systemic Amyloidosis Manifesting as a Rare Cause of Hepatic Failure

Hung Hsu Hung, De Feng Huang, Cheng Hwai Tzeng, Cheng Hsi Su, Tung Ping Su, Hung Chieh Chen, Shyh Haw Tsay, Han Chieh Lin, Jaw Ching Wu, Shou Dong Lee, Chien Wei Su*


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7 引文 斯高帕斯(Scopus)


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.

頁(從 - 到)161-165
期刊Journal of the Chinese Medical Association
出版狀態Published - 3月 2010


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