非硬化性门脉高压性肝脏病变的病理特征  

Pathological features of several non-cirrhotic portal hypertensive liver lesions

作　　者：刘晖[1] 孔贺利[1] 宋晨朝[1] 李磊 丁惠国 Liu Hui;Kong Heli;Song Chenzhao;Li Lei;Ding Huiguo(Department of Pathology,Beijing Youan Hospital,Capital Medical University,Beijing 100069,China)

机构地区：[1]首都医科大学附属北京佑安医院病理科,100069 [2]首都医科大学附属北京佑安医院肝病消化中心,100069

出　　处：《北京医学》2019年第10期884-887,共4页Beijing Medical Journal

基　　金：国家科技重大专项“艾滋病和病毒性肝炎等重大传染病防治”科技重大专项(2017ZX10203202003008);北京市医院管理局——“扬帆”计划(ZYLX201610);北京市医院管理局“登峰”人才培养计划(DFL20150602);北京市卫生系统高层次卫生人才(2013-3-073)

摘　　要：目的从病理学角度阐明门脉高压症相关的几种非硬化性肝脏疾病的特点。方法检索2007年1月至2017年12月首都医科大学附属北京佑安医院病理科接收临床诊断为"肝硬化原因待查",病理诊断为"非硬化性门脉高压症、肝门脉硬化、特发性门脉高压症、结节性再生性增生、先天性肝纤维化或系统性疾病累及肝"的病例共134例,所有病例的组织切片均行常规HE染色、Masson trichrome染色、Reticulin染色,光镜下观察病变特点,总结其病理特征。结果所有病例的组织切片均未见肝硬化组织学改变。非硬化性门脉高压症、肝门脉硬化及特发性门脉高压症病变相似,以汇管区间质纤维化、门脉支管腔缩小或闭塞、门脉小支向邻近肝实质扩张为特点;实质内有时见肝窦扩张(巨大肝窦)及异常扩张的静脉腔,病变进展可见结节性再生性增生。先天性肝纤维化为胆管板发育不良所致,常致门脉高压或胆管炎,而非真正意义的肝硬化。系统性疾病如淀粉样变性、骨髓增生性疾病,可见淀粉样物、髓外造血细胞阻塞或浸润肝窦,也可致门脉高压症。结论肝活检对于门脉高压症的诊断十分必要,既可排除肝硬化,又可明确引起非硬化性门脉高压症的病因。Objective To elucidate the pathological features of several common diseases associated with portal hypertension(PHT)in order to help clinicians understand such lesions from a pathological point of view.Methods The 134 cases pathologically diagnosed as non-cirrhotic portal hypertension(NCPH),hepatoportal sclerosis,idiopathic portal hypertension(IPH),congenital hepatic fibrosis(CHF),systemic diseases involving the liver were retrieved in Department of Pathology,Beijing Youan Hospital,Capital Medical University from January 2007 to December 2017.All the tissue slices were performed by HE staining,special staining for Masson trichrome and Reticulin were observed under light microscope.Their pathological features were summarized combined with clinical features and imaging data.Results Histological features of cirrhosis were not found in all cases.NCPH,hepatoportal sclerosis and IPH had the same pathological changes:luminal narrowing,sclerosis,or disappearance of the portal vein(PV)were the key histological changes in the portal tract(PT)which might show fibrosis without inflammation.The small branch of the PV dilated and herniated into the adjacent liver parenchyma.Sometimes the sinusoidal dilatation(megasinusoids)was seen in the parenchyma.CHF caused by ductal plate malformation,often resulted in PHT or cholangitis,but it was not true cirrhosis.Systemic disease,such as amyloidosis and myeloproliferative disorders,could also result in PHT.Conclusions Liver biopsy is essential for diagnosis of PHT,which not only can rule out cirrhosis,but also be able to confirmed the cause of NCPH.

关 键 词：门脉高压症 非硬化性 肝 病理学 

分 类 号：R73[医药卫生—肿瘤]