慢性活动性EB病毒性肝炎的临床病理特征  

Clinicopathologic features of chronic active Epstein-Barr virus hepatitis

作　　者：李鑫静[1] 曲利娟[1] 郑雄伟[2] 陈丽红[3] 董菁[4] 李东良[5] 潘晨[6] 吕旭江[6] 郑智勇[1] 

机构地区：[1]中国人民解放军南京军区福州总医院病理科,福建省福州市350025 [2]福建省肿瘤医院病理科,福建省福州市350014 [3]福建医科大学病理系,福建省福州市350004 [4]福建医科大学附属第一医院肝病中心肝内科,福建省福州市350005 [5]福建医科大学福总临床医学院(南京军区福州总医院)肝胆内科,福建省福州市350025 [6]福州市传染病医院,福建省福州市350025

出　　处：《世界华人消化杂志》2013年第23期2325-2330,共6页World Chinese Journal of Digestology

摘　　要：目的:探讨慢性活动性EB病毒性肝炎(chronic active Epstein-Barr virus hepatitis,CAEBVH)的临床病理特征、诊断、鉴别诊断、治疗方法和预后.方法:报道2例CAEBVH,总结其临床表现、病理特征、诊断及鉴别诊断、治疗及预后,并结合文献进行分析讨论.结果:两例患者为青少年男性,例1主要表现为发热、黄疸、肝脾肿大、肝功能异常,例2首先以血生化检查肝功能异常为特征,后期出现双下肢水肿、尿黄、乏力、脾大,伴中枢神经系统症状.活检肝组织于光镜下观察:两例均出现不同程度的肝细胞大泡性脂肪变性和纤维组织增生,肝小叶内可见点灶状坏死,肝窦内成串淋巴细胞浸润,界板炎和汇管区炎症.电镜观察:慢性肝炎,肝细胞脂肪变性及纤维增生,未见髓鞘样小体、特征性溶酶体、乙肝表面抗原等特征性病变结构.EBER原位杂交均检出细胞核阳性的淋巴细胞.例1病程为2年零4 mo,例2病程为13年零5 mo,最终均死亡.结论:慢性活动性EB病毒感染肝炎的临床表现不具有特异性,易于误诊或漏诊.病理学特点为大泡性脂肪变,肝窦内成串淋巴细胞浸润,肝小叶点灶状坏死,界板炎和汇管区炎症,以及EBER原位杂交均检出细胞核阳性的淋巴细胞.该病预后差,早期诊断是治疗的关键.AIM： To investigate the clinicopathologic fea- tures of chronic active Epstein-Barr virus hepatitis （CAEBVH） as well as its diagnosis, differen- tial diagnosis, treatment and prognosis. METHODS： We presented the clinical mani- festations, histopathological characteristics, diagnosis, treatment and prognosis of two cases of CAEBVH. A literature review was also per- formed to summarize the characteristics of this clinical entity. RESULTS： Of two young male patients, one presented with intermittent fever, jaundice, hep- atosplenomegaly and abnormal liver function, the other had abnormal liver biochemical tests and symptoms including edema of lower limbs, yellowish urine, fatigue, splenomegaly, and cen- tral nervous system symptoms. Histopathologic examination of liver biopsies revealed varying degrees of macrovesicular steatosis and fibrosis, scattered lobular necrosis, beaded sinusoidal lymphocytic infiltration, portal inflammation and interface activity. Electron microscopic in- vestigation showed chronic hepatitis along with steatosis of liver cells and fibrosis. No mylinoid body, special lysosome, glycogen storage or hepatitis B surface antigens were found. In situ hybridization （ISH） for EBV early RNA （EBER） showed EBER-positive nuclei of lymphocytes. Both patients ended in death. The course was 2 years and 4 months for case I and 13 years and 5 mo for case 2. CONCLUSION： CAEBVH has no specific clinical features, which makes it easy to reach a misdiag- nosis. Pathologic features include macrovesicu- lar steatosis, fibrosis, beaded sinusoidal lympho- cytes infiltration, scattered lobular necrosis, in- terface activity and portal inflammation. EBER- positive nuclei of lymphocytes can be detected by ISH. This disease has a poor prognosis and early diagnosis is pivotal for appropriate clinical management.

关 键 词：EB病毒 慢性感染 病毒性肝炎 中枢神经系统症状 

分 类 号：R575.1[医药卫生—消化系统]