Thomas R M, Schiano T D, Kueppers F, Black M
Department of Pathology, Temple University School of Medicine, Philadelphia, PA, USA.
Hum Pathol. 2000 May;31(5):575-7. doi: 10.1053/hp.2000.6685.
Alpha1-antichymotrypsin (A1AC) is an acute phase serine protease inhibitor, similar to alpha1-antitrypsin (A1AT) in amino acid sequence. A1AT deficiency is known to be associated with emphysema and cirrhosis; deficiency of serum A1AC has been reported to be associated with emphysema, childhood asthma, and cryptogenic cirrhosis. The hepatocyte globules associated with A1AT deficiency have been well described; A1AC deficiency also has been reported to be associated with hepatocyte globules. The aim of this study was to describe the globules of A1AC and to compare them with A1AT globules. Immunohistochemistry for A1AC and A1AT was performed on liver biopsy specimens from 15 hepatitis C virus (HCV)-positive cirrhotic patients, 14 non-HCV cirrhotic patients, and 12 other patients with chronic hepatitis C but no cirrhosis, all of whom had known serum levels of A1AC; most had known serum levels of A1AT. Five of 15 HCV-positive cirrhotic patients, 1 of 14 non-HCV cirrhotic patients, and 1 of 12 noncirrhotic chronic hepatitis C patients had A1AC globules. Two of 15 HCV-positive cirrhotic patients and 2 of 14 non-HCV cirrhotic patients had A1AT globules. Histologically, the globules of A1AC were similar to those of A1AT but were smaller and fewer; the PAS/D stain was not as helpful for A1AC as it was for A1AT; immunohistochemistry was most useful. There was not a good correlation between serum levels of A1AC and its globules in hepatocytes. A1AC globules should be included in the differential diagnosis of hepatocyte inclusions.
α1-抗糜蛋白酶（A1AC）是一种急性期丝氨酸蛋白酶抑制剂，其氨基酸序列与α1-抗胰蛋白酶（A1AT）相似。已知A1AT缺乏与肺气肿和肝硬化有关；据报道，血清A1AC缺乏与肺气肿、儿童哮喘和隐源性肝硬化有关。与A1AT缺乏相关的肝细胞小球已得到充分描述；据报道，A1AC缺乏也与肝细胞小球有关。本研究的目的是描述A1AC小球，并将其与A1AT小球进行比较。对15例丙型肝炎病毒（HCV）阳性肝硬化患者、14例非HCV肝硬化患者和12例其他慢性丙型肝炎但无肝硬化患者的肝活检标本进行了A1AC和A1AT的免疫组织化学检测，所有患者均已知血清A1AC水平；大多数患者已知血清A1AT水平。15例HCV阳性肝硬化患者中有5例、14例非HCV肝硬化患者中有1例、12例非肝硬化慢性丙型肝炎患者中有1例有A1AC小球。15例HCV阳性肝硬化患者中有2例、14例非HCV肝硬化患者中有2例有A1AT小球。组织学上，A1AC小球与A1AT小球相似，但更小、数量更少；PAS/D染色对A1AC的帮助不如对A1AT的大；免疫组织化学最有用。肝细胞中A1AC血清水平与其小球之间没有良好的相关性。A1AC小球应纳入肝细胞内含物的鉴别诊断中。
