关键词: 肝淀粉样变性, 磁共振, 体层摄影术, 扩散加权成像

Abstract: Objective To analyze the clinical and imaging features of 12 patients with hepatic amyloidosis.Methods Data from 12 patients who had undergone pathological biopsies between February 2017 and February 2023 were collected and analyzed. General demograpgics, clinical findings, laboratory results and imaging findings were included in this study.Results In 12 patients diagnosed with hepatic amyloidosis, the predominant clinical manifestations included abdominal distension, poor appetite, and abnormal liver function. Among these patients, 10 showed elevated alkaline phosphatase(ALP), 11 had elevated glutamyltransferase(GGT), 7 exhibited decreased albumin(ALB), 5 tested negative for tumor markers, 4 had elevated CA125, and 6 indicated elevated CA199 levels. Hepatomegaly was evident in all 12 patients, Additionally, an enlarged spleen was identified in 7 patients, abdominal effusion in 7, and 9 displayed a “frozen snowflakes on window”-like patchy low-density or low signal enhancement in the liver parenchyma during both the portal and delayed phases.Conclusion Hepatic amyloidosis predominantly affects middle-aged and elderly women. The primary clinical symptoms include abdominal distension and abnormal liver function. Most patients exhibit elevated levels of ALP and GGT, with some showing abnormal some tumor markers. The most frequent imaging findings are enlargement of the liver and spleen, ascites, heterogeneous liver parenchymal enhancement, and enhancement patteins reminiscent of “frozen snowflakes on a window”. The definitive diagnosis of hepatic amyloidosis relies on a combination of imaging results and pathological findings from liver puncture biopsy.

Key words: Hepatic amyloidosis, Magnetic resonance imaging, Tomography, Diffusion-weighted imaging