title: Primary hepatic amyloidosis: a mini literature review and five cases report.
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.
authors.affiliation: Department of Infectious Disease, Third Affiliated Hospital of Hebei Medical University, Shijiazhuang, China.
authors.forename: Ya-Dong
authors.initials: YD
authors.lastname: Wang
authors.forename: Cai-Yan
authors.initials: CY
authors.lastname: Zhao
authors.forename: Hong-Zhu
authors.initials: HZ
authors.lastname: Yin
mesh_terms: D000686:Amyloidosis / Q000150:complications / Q000175:diagnosis / Q000453:epidemiology / Q000276:immunology / Q000628:therapy; D015415:Biomarkers / Q000032:analysis; D003937:Diagnosis, Differential; D006801:Humans; D007147:Immunoglobulin Light Chains / Q000032:analysis; D000075363:Immunoglobulin Light-chain Amyloidosis; D008099:Liver / Q000276:immunology / Q000473:pathology; D008107:Liver Diseases / Q000150:complications / Q000175:diagnosis / Q000453:epidemiology / Q000276:immunology / Q000628:therapy; D008558:Melphalan / Q000008:administration & dosage; D011237:Predictive Value of Tests; D033581:Stem Cell Transplantation; D016896:Treatment Outcome
chemical_list: D015415:Biomarkers; D007147:Immunoglobulin Light Chains; D008558:Melphalan