Resumen

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.

Palabras clave: Amyloidosis immunoglobulin light chains liver disease stem cell transplantation.

2012-08-21   |   578 visitas   |   Evalua este artículo 0 valoraciones

Vol. 11 Núm.5. Septiembre-Octubre 2012 Pags. 721-727 Ann Hepatol 2012; 11(5)