Roggli V L, Hausner R J, Askew J B
Am J Clin Pathol. 1981 Apr;75(4):538-42. doi: 10.1093/ajcp/75.4.538.
Percutaneous needle biopsy specimens of the liver from three elderly persons (aged 77, 71, and 66) demonstrated eosinophilic intracytoplasmic globules within hepatocytes, particularly in the periportal and periseptal areas. These globules were periodic acid-Schiff positive and diastase resistant, and were identified as alpha-1-antitrypsin by immunofluorescence technics. Two of the patients had cirrhosis, and identification of protease inhibitor (Pi) type by acid starch electrophoresis and crossed immunoelectrophoresis demonstrated SZ and MZ genotypes. The patient with SZ genotype also had a long history of chronic obstructive pulmonary disease. Pi-typing was not performed for the third patient, who did not have cirrhosis. The morphologic identification of alpha-1-antitrypsin disease in liver biopsies of persons of any age is important because of (1) possible multisystem involvement (hepatic and pulmonary), (2) increased frequency of hepatocellular carcinoma, and (3) implications for genetic counseling for other family members.
对三名老年人（年龄分别为77岁、71岁和66岁）的肝脏进行经皮针吸活检，结果显示肝细胞内存在嗜酸性胞浆内小球，尤其是在汇管区和间隔周围区域。这些小球对过碘酸-希夫染色呈阳性且耐淀粉酶，通过免疫荧光技术鉴定为α1-抗胰蛋白酶。其中两名患者患有肝硬化，通过酸性淀粉凝胶电泳和交叉免疫电泳鉴定蛋白酶抑制剂（Pi）类型，显示为SZ和MZ基因型。具有SZ基因型的患者还有慢性阻塞性肺疾病的长期病史。第三名没有肝硬化的患者未进行Pi分型。由于（1）可能存在多系统受累（肝脏和肺部）、（2）肝细胞癌的发病率增加以及（3）对其他家庭成员进行遗传咨询的意义，因此在任何年龄人群的肝脏活检中对α1-抗胰蛋白酶病进行形态学鉴定都很重要。
