| Journal: | Annals of hepatology |
| Database: | PERIÓDICA |
| System number: | 000419068 |
| ISSN: | 1665-2681 |
| Authors: | Ya-Dong, Wang1 Cai-Yan, Zhao1 Hong-Zhu, Yin1 |
| Institutions: | 1Hebei Medical University, Third Affiliated Hospital, Shijiazhuang, Hebei. China |
| Year: | 2012 |
| Season: | Sep-Oct |
| Volumen: | 11 |
| Number: | 5 |
| Pages: | 721-727 |
| Country: | México |
| Language: | Inglés |
| Document type: | Artículo |
| Approach: | Caso clínico |
| English 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 |
| Disciplines: | Medicina |
| Keyword: | Gastroenterología, Inmunología, Amiloidosis hepática primaria, Inmunoglobulinas, Trasplante de células madre |
| Keyword: | Gastroenterology, Immunology, Primary hepatic amyloidosis, Immunoglobulins, Stem cells transplantation |
| Full text: | Texto completo (Ver PDF) |
