Primary hepatic amyloidosis:report of 4 cases and reviews of the literature in and abroad
- VernacularTitle:肝淀粉样变4例报告并国内文献分析
- Author:
Lixin YANG
;
Hong LU
;
Jiaming QIAN
- Publication Type:Journal Article
- Keywords:
Primary hepatic amyloidosis;
Clinical characteristics;
Retrospective studies
- From:
Chinese Journal of Practical Internal Medicine
2001;0(03):-
- CountryChina
- Language:Chinese
-
Abstract:
Objective To comprehend the clinical characteristics and treatment of primary hepatic amyloidosis.Methods A total of 10 cases of primary hepatic amyloidosis included. 4 cases from Peking Union Medical College Hospital and 6 cases from other hospitals.reported in the literature in recent 20 years were reviewed,Results (1)The frequent clinical symptoms related to primary hepatic amyloidosis presented as hepatomegaly(90%),abdominal distension(80%),shifting dullness(60%),anorexia(50%),fatigue(40%),edema(40%),weight loss(40%) and abdominal pain(30%).(2)Laboratory tests revealed elevated serum alkaline phosphatase which was (558.3?517.2)U/L,and other liver function involvement were rare:six patients(85.7%)had either a serum or urine monoclonal protein.(3)Liver biopsy is golden standard.The amyloid deposition was mainly located at sinusoida(4 casese,57.1%),and only 2 cases was at vascular(28.6%).Complication of bleeding after liver biopsy was reported(1 case).(4)The classical therapy scheme was melphalan and prednisone,four cases died from liver function failure and infection.Conclusion The clues to the diagnosis of primary hepatic amyloidosis include elevated monoclonal protein,hepatomegaly and an unexplained elevated serum alkaline phosphatase level which is not parallel with other liver functions.Live biopsy is golden standard.The treatment scheme include melphalan and prednisone,but its prognosis is poor.
