摘要
目的总结原发性肝脏淀粉样变性患者的临床特点,提高对本病的认识,减少误诊。方法回顾性分析2007-2016年收治的7例原发性肝脏淀粉样变性患者的临床资料并复习相关文献。结果 7例患者临床表现主要为腹胀和食欲不振;肝功能检查γ-谷氨酰转肽酶(GGT)和碱性磷酸酶(ALP)升高明显;影像学提示肝脏均匀性增大。肝脏和胃部病理检查提示淀粉样物质沉积,刚果红染色阳性。1例患者肝脏穿刺后出血抢救无效死亡。4例未给予化疗,均因各种并发症死亡。1例给予马法兰联合泼尼松治疗,7个月后死亡。1例给予硼替佐米联合地塞米松治疗,治疗8个月病情稳定。结论原发性肝脏淀粉样变性临床少见,肝脏均匀性增大,肝功能以GGT和ALP升高为主,病理检查是金标准。一般预后差,如果早期发现,可尝试给予新型蛋白酶体抑制剂化疗。
Objective To summarize the clinical features of patients diagnosed as primary hepatic amyloidosis, and to improve the current understanding of the disease and decrease misdiagnosis. Methods The clinical data of 7 prima- ry hepatic amyloidosis patients during 2007 to 2016, were retrospectively analyzed and the literatures were reviewed. Re- suits Abdominal distension and poor appetite were the most common clinical symptoms in 7cases. GGT and ALP severe- ly elevated. Imaging studies indicated liver enlargement as the common imagine presentation. Liver and stomach patholog- ic examinations showed amyloid deposition and Congo red staining was positive. One patient died of bleeding after liver biopsy. Four cases were not given chemotherapy and died of a variety of complications. One case received the treatment with melphalan plus prednisone, but still died 7 months later. One case had stable condition after bortezomib plus dexa- methasone therapy for about 8 months. Conclusion Primary hepatic amyloidosis is rare. Laboratory tests have showed el- evated GGT and ALE Liver biopsy is still the gold standard. The chemotherapy ofproteasome inhibitors should be consid- ered if it is found early although the poor prognosis and limited treatment of amyloidosis.
出处
《北京医学》
CAS
2016年第9期873-876,F0002,共5页
Beijing Medical Journal
关键词
肝脏淀粉样变性
诊断
治疗
hepatic amyloidosis
diagnosis
therapy
