A 60-year-old woman, who had had appetite loss and general malaise, was recognized cholestatic disorders, and was admitted to a hospital. She was diagnosed as primary amyloidosis (AL-type) by liver biopsy. Her general condition was rapidly aggravated and she was transferred to our hospital, when she had grade II encephalopathy and PT was 33%. Artificial liver support was introduced and hepatic coma was transiently recovered, but liver failure developed again and she finally died. Autopsy revealed marked hepatomegaly, and massive deposition of amyloid in the liver. Amyloid deposition was also found in the multiple organs, especially marked in the spleen, kidney, thyroid gland and adrenal gland. Primary amyloidosis with hepatic dysfunction, as shown in this case, is rare and the prognosis is generally poor, but the early diagnosis is important, and chemotherapy should have considered in this case in her earlier phase of this disease.
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