Hepatic amyloidosis disease: clinic analysis of six cases / 中国医师进修杂志

ABSTRACT

Objective To summarize the clinical characteristics of hepatic amyloidosis disease by analyzing the data of this disease. Methods Six cases of hepatic amyloidosis out of 32 cases of amyloidosis were selected and the patients clinical characteristics, laboratory tests, treatments and prognosis were analyzed retrospectively. Results All the patients presented weight loss, fatigue, hepatomegaly as well as proteinuria. Four patients had splenomegaly and 3 patients had ventricular hypertrophy. The levels of alkaline phosphatase(ALP)and γ-glutamyl transpeptidase(GGT)in all patients elevated 1.5 times more than the upper limit of normal.Three of them performed immunoglobulin electrophoresis,and the results of which were abnormal.All of them did involved organ biopsy,including liver(3 cases)and kidney(3 cases).All of them demonstrated amyloid deposition.None of them suffered bleeding and death during the biopsy. Organ pathology assessing was not enough. Prognostic markers included hyperbilirubinemia,ALP more than 500 U/L,B type natriuretic peptides,plasma cells clone,β2 microglobulin and other organ function. Conclusions The patients with hepatic amyloidosis disease present hepatomegaly with different sizes and mildly abnormal liver test(mainly with elevated GGT and ALP). Most cases have multiple organs pathology including kidney, spleen and heart. Laboratory test often shows hyperlipidemia and abnormal serum free light chain.It is always confirmed by pathology.