肝淀粉样变影像学表现

Imaging manifestations of hepatic amyloidosis

目的观察肝淀粉样变的影像学表现。方法回顾性分析11例病理确诊的肝淀粉样变患者的临床、影像及病理学资料,观察肝淀粉样变的特征性影像学表现。结果7例患者接受CT扫描,平扫CT表现为肝脏体积弥漫性增大、肝实质密度不均匀减低;其中5例接受增强CT扫描,动脉期肝实质见斑片状强化或未见异常强化,脾脏轻度均匀强化,门脉期及延迟期见肝实质内大片状“窗凌花”样强化减低区,脾脏强化程度稍减低;2例动脉期示双肾皮质强化程度减低,皮髓质分界不清。8例接受MR扫描,肝脏体积明显增大,边缘光滑,肝实质T1WI信号均匀、T2WI稍均匀增高,肝纹理明显减少;脾脏体积正常或稍大,信号正常或均匀减低;其中3例接受增强扫描,门脉期及延迟期见肝实质内弥漫斑片状“窗凌花”样强化减低区;2例门脉期及延迟期脾脏强化程度稍减低;1例动脉期见双肾皮质强化程度减低。5例接受肝脏瞬时弹性超声成像,所测肝实质硬度值均≥75 kPa。结论肝淀粉样变影像学表现具有一定特征性,有助于早期诊断、监测病情及评估疗效。

Objective To observe imaging manifestations of hepatic amyloidosis.Methods Clinical,imaging and pathological data of 11 patients with hepatic amyloidosis diagnosed pathologically were retrospectively analyzed,and the characteristic imaging manifestations were observed.Results In 7 patients underwent plain CT scanning,diffuse enlargement of liver and uneven decrease of liver parenchyma density were noticed.Among them,5 cases underwent enhanced CT scanning,patchy enhancements or no enhancement of liver parenchyma both existed,and spleens were found with mild homogeneous arterial phase enhancements,while in portal phase and delay phase,large areas of reduced enhancement like“frozen snowflakes on window”of liver parenchyma,and slightly decreased enhancement degree of spleen were observed.Decreased enhancement of bilateral renal cortex and unclear boundary between cortex and medulla in arterial phase were noticed in 2 cases.In 8 patients underwent MR scanning,the liver volumes significantly increases with smooth edges and uniform signals on T1WI and slightly high signals on T2WI,and liver texture decreased significantly.Spleen volume was normal or slightly increased,with normal or evenly decreased signals.Among them,3 cases underwent enhanced MR scanning,liver parenchyma were found enhanced like“frozen snowflakes on window”in portal phase and delay phase.The enhancement of spleen slightly decreased in portal phase and delay phase in 2 cases,while enhancement of bilateral renal cortex decreased in arterial phase in 1 case.Transient elastography of liver was performed in 5 patients,and the hardness of liver parenchyma was≥75 kPa.Conclusion Imaging manifestations of hepatic amyloidosis had some characteristics,which were helpful for early diagnosis,monitoring and evaluation on curative effect of this disease.