Primary hepatic amyloidosis: a mini literature review and five cases report



Título del documento: Primary hepatic amyloidosis: a mini literature review and five cases report
Revista: Annals of hepatology
Base de datos: PERIÓDICA
Número de sistema: 000419068
ISSN: 1665-2681
Autores: 1
1
1
Instituciones: 1Hebei Medical University, Third Affiliated Hospital, Shijiazhuang, Hebei. China
Año:
Periodo: Sep-Oct
Volumen: 11
Número: 5
Paginación: 721-727
País: México
Idioma: Inglés
Tipo de documento: Artículo
Enfoque: Caso clínico
Resumen en inglés 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
Disciplinas: Medicina
Palabras clave: Gastroenterología,
Inmunología,
Amiloidosis hepática primaria,
Inmunoglobulinas,
Trasplante de células madre
Keyword: Gastroenterology,
Immunology,
Primary hepatic amyloidosis,
Immunoglobulins,
Stem cells transplantation
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