肝脏淀粉样变性6例临床分析并文献复习

Clinical characteristics of 6 cases with hepatic amyloidosis and literature review

通过对6例经肝、肾组织学检查诊断为肝脏淀粉样变性患者的临床资料、实验室检查(肝功能、血脂、尿常规及血常规等)、肝脏超声和CT等影像学检查及肝肾组织病理学检查等进行回顾性分析,以提高临床医师对肝脏淀粉样变性的认识.结果表明,6例患者以乏力、腹胀、纳差等消化道症状为主,碱性磷酸酶(alkaline phosphatase,ALP)和γ-谷氨酰转肽酶(gamma-glutamyl transpeptidase,γ-GGT)显著升高;部分患者表现为低白蛋白血症、高脂血症及蛋白尿,血清可见M蛋白,尿中可检出BJ蛋白.肝肾组织病理可见均染的粉红色物质沉积,刚果红染色阳性.6例患者中4例为肝脏淀粉样变性合并肾淀粉样变,其中2例患者同时合并多发性骨髓瘤(mutiple myeloma,MM).提示肝脏淀粉样变性患者常合并其他器官受累,临床表现多样,误诊率高.肝肾组织学检查及特异性组织学染色(刚果红染色)有助于肝脏淀粉样变性的早期诊断.

The clinical data, laboratory test (liver function, blood lipid, urine routine and blood routine), liver ultrasound, CT, and the pathological examination of liver and kidney of 6 cases with hepatic amyloidosis who were diagnosed by liver and kidney histology were analyzed retrospectively, so as to improve the understanding of clinical doctors on hepatic amyloidosis. The clinical manifestations of 6 patients included fatigue, abdominal distention and poor appetite, the level of alkaline phosphatase (ALP) and gamma-glutamyl transpeptidase (γ-GGT) were elevated significantly. Some patients showed hypoalbuminemia, hyperlipidemia and urinary protein, M protein could be detected in serum and BJ protein could be detected in urine. The histopathology of liver and kidney showed a large amount of pink material deposition and Congo red staining was positive. Four of the six cases had hepatic amyloidosis and renal amyloidosis, among whom 2 cases were with multiple myelomas (MM). Patients with hepatic amyloidosis often involved in other organs, with diverse clinical manifestations and high misdiagnosis rate. Liver and kidney histological examination and specific histologic staining (Congo red staining) can be used to diagnose liver amyloidosis in patients with high suspicion of liver amyloidosis and to prove guidance for clinical treatment.