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• W1567163361 abstract "Primary hepatic amyloidosis (PHA) is characterized by abnormal deposition of monoclonal immunoglobulin light chains (AL) in the liver. This rare condition is frequently undiagnosed or misdiagnosed and can be associated with poor prognosis. At present, the precise pathogenesis is not fully understood. Despite that hepatomegaly and elevated alkaline phosphatase (ALP) are present in most patients with PHA, no specific clinical markers have been identified. Staining of hepatic tissues with Congo Red is often regarded as the “gold standard”. Pharmacological therapy should aim to rapidly reduce the supply of misfolded amyloidogenic AL. High-dose intravenous melphalan (HDM) and autologous stem cell transplantation (ASCT) appear to be the most appropriate therapy but controversies still exist." @default.
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• W1567163361 date "2012-09-01" @default.
• W1567163361 modified "2023-10-01" @default.
• W1567163361 title "Primary hepatic amyloidosis: a mini literature review and five cases report" @default.
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• W1567163361 doi "https://doi.org/10.1016/s1665-2681(19)31450-4" @default.
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