特发性非肝硬化性门静脉高压症的临床及病理特点分析

摘要/Abstract

摘要： 目的探讨特发性非肝硬化性门静脉高压症(INCPH)的临床及病理特点。方法回顾性分析44例患者的临床资料,其中23例为INCPH,21例为病毒性肝炎失代偿期肝硬化,比较二者之间的差异,同时分析23例INCPH患者的病理特点。结果 INCPH组以女性(60.87%)为主,年龄较轻(39.8±15.4)岁,而病毒性肝炎失代偿期肝硬化组以男性(80.95%)为主,年龄较大(53.3±11.2)岁,差异均有统计学意义(P<0.05)。INCPH组以消化道出血(39.13%,9/23)为主要就诊症状,而病毒性肝炎失代偿期肝硬化组以腹水(85.71%,18/21)为主要就诊症状。与INCPH组相比,病毒性肝炎失代偿期肝硬化组红细胞(RBC)、血小板(PLT)、凝血酶原活动度(PT)、白蛋白(Alb)、胆碱酯酶(CEA)明显降低[RBC:(3.79~4.14)×10¹²/L比(3.33~3.94)×10¹²/L,PLT:(91~235)×10⁹/L比(41~72)×10⁹/L,PT:(66.5~95)%比(49.60~65.00)%,Alb:(37~39)g/L比(26~33)g/L,CEA:(4596~6408)U/L比(2368~3497.5)U/L,均P<0.05];而凝血国际标准化比值(INR)、丙氨酸氨基转移酶(ALT)、门冬氨酸氨基转移酶(AST)、总胆红素(TBil)、肝脏硬度值(LSM)、肝功能评分(CTP)明显高于INCPH组 [INR:(1.02~1.19)比(1.19~1.42),ALT:(15~21)U/L比(17.50~36.50)U/L,AST:(21~31)U/L比(32~57)U/L,TBil:(10.8~18.3)μmol/L比(20.05~43.05)μmol/L,LSM:(5.90~13.3)kPa比(17.60~24.85)kPa,CTP:(5~7)比(7~9),均P<0.05], 但白细胞(WBC)、血红蛋白(HGB)两组比较无差异(P>0.05)。同时两组比较,INCPH组有明显脾脏增厚、门静脉结构紊乱、门静脉海绵样变性、侧支循环建立, 差异均有统计学意义[脾厚:(51~79)mm比(49~59)mm,门静脉结构紊乱:34.78%比4.76%,门静脉海绵样变性:39.13%比4.76%,侧支循环建立:65.22%比33.33%,P<0.05],但门静脉直径、脾静脉直径、脾静脉直径/门静脉直径、脾大发生率、门脉高压性胃病发生率以及食管和胃底静脉曲张程度均无差异(P>0.05)。23例INCPH患者的肝组织病理主要表现为肝细胞排列基本正常、无假小叶形成、门静脉未见血栓形成、小叶内静脉及中央静脉扩张、汇管区扩张、肝窦扩张、肝细胞区域水样变。结论 INCPH与病毒性肝炎失代偿期肝硬化的临床表现、实验室检查、影像学指标存在一定的差异,但二者之间也存在一定的相似性,因此诊断时仍需结合肝脏病理检查。

关键词: 特发性非肝硬化性门静脉高压症, 病毒性肝炎, 失代偿期肝硬化, 病理

Abstract: Objective To compare the clinical parameters between idiopathic non-cirrhotic portal hypertension (INCPH) and virus hepatitis-related decompensated cirrhosis, and to analyze clinical and pathological features of INCPH. Methods Clinical data of 44 patients, including 23 patients with INCPH and 21 with virus hepatitis-related decompensated cirrhosis, were retrospectively analyzed. The clinical parameters were compared between the 2 groups, and the pathological characteristics of INCPH were investigated. Results The patients in INCPH group were younger (39.83 ± 15.38 years) and predominantly female (60.87%) , while the patients in decompensated cirrhosis group were older (53.33 ± 11.24 years) and predominantly male (80.95%), and the differences were statistically significant (P<0.05). The main symptom was gastrointestinal bleeding (39.13%) in INCPH group, and ascites (85.71%) in the other group (P<0.05). Red blood cell, platelet, prothrombin activity, albumin, cholinesterase in virus hepatitis-related decompensated cirrhosis group were significantly lower than those in INCPH group (P<0.05), while international normalized ratio, alanine transaminase, aspartate transaminase, total bilirubin, liver stiffness measurement and Child-Turcotte-Pugh score were significantly higher than those in INCPH group (P<0.05). And there was no significant difference in white blood cell or hemoglobin between the 2 groups (P>0.05). Compared with virus hepatitis-related decompensated cirrhosis group, INCPH group had thicker spleen, more portal vein structure disorder, more portal vein cavernous degeneration, and more collateral circulation, and the differences were statistically significant (P<0.05). However, there were no significant differences in portal vein diameter, splenic vein diameter, splenic vein diameter/portal vein diameter, incidence of splenomegaly, incidence of portal hypertensive gastropathy or degree of esophageal and gastric varices between the 2 groups (P>0.05). The liver histopathology of INCPH mainly showed that there was no obvious disorder of hepatocyte arrangement, formation of pseudo-lobe, or thrombosis in portal vein, and that there was venectasia of interlobular vein and central vein, expansion of portal area, dilation of liver sinuses, and watery degeneration of liver cells. Conclusion There were both differences and similarities in clinical manifestations, laboratory tests, and imaging indicators between INCPH and virus hepatitis-related decompensated cirrhosis, so the liver pathological examination is necessary for diagnosis.

Key words: Idiopathic non-cirrhotic portal hypertension, Virus hepatitis-related decompensated cirrhosis, Clinical, Pathology

王晴晴, 彭宇辉, 唐艳芳. 特发性非肝硬化性门静脉高压症的临床及病理特点分析[J]. 肝脏, 2021, 26(3): 291-295.

WANG Qing-qing, PENG Yu-hui, TANG Yan-fang. Analysis of clinical and pathological features of idiopathic non-cirrhotic portal hypertension[J]. Chinese Hepatolgy, 2021, 26(3): 291-295.

参考文献

[1] Choksi V, Chokshi B, Chu A, et al. Gastric varices in absence of splenic vein thrombosis: a rare entity of idiopathic non-cirrhotic portal hypertension. Cureus. 2017, 9: e1179.
[2] Riggio O, Gioia S, Pentassuglio I, et al. Idiopathic noncirrhotic portal hypertension: current perspective. Hepat Med, 2016, 8:81-88.
[3] 赵连晖,贾继东. 特发性非肝硬化性门脉高压.肝脏,2019, 24: 219-220.
[4] European Association for the Study of the Liver. EASL clinical practice guidelines: vascular diseases of the liver. J Hepatol, 2016, 64: 179-202.
[5] 中华医学会肝脏病学分会.肝硬化诊治指南.临床肝胆病杂志,2019, 35:2408-2418.
[6] Schouten JNL, Verheij J, Seijo S. Idiopathic non-cirrhotic portal hypertension: a review. Orphanet J Rare Dis, 2015, 10:67.
[7] Khanna R, Sarin SK. Non-cirrhotic portal hypertension-di-agnosis and management . J Hepatol, 2014, 60: 421-441.
[8] Goel A, Elias JE, Eapen CE, et al. Idiopathic NonCirrhotic Intrahepatic Portal Hypertension (NCIPH)-newer insights into pathogenesis and emerging newer treatment options. J Clin Exp Hepatol, 2014, 4: 247-256.
[9] Guido M, Sarcognato S, Sonzogni A, et al. Obliterative portal venopathy without portal hypertension:an underestimated condition. Liver Int, 2016, 36: 454-460.
[10] 何创业, 吕勇, 陈辉, 等. 瞬时弹性成像技术在诊断特发性门静脉高压症中的价值.中华肝脏病杂志. 2018, 26: 310-312.
[11] 刘海博,朴秉国,刘静.特发性非肝硬化门静脉高压症影像学研究进展. 人民军医.2017, 62: 658-661.
[12] 马雪梅, 任辉, 金波, 等. 21例特发性门脉高压临床及病理特点分析. 传染病信息. 2017, 30: 168-171.
[13] 刘海博, 张博静, 吕勇, 等.特发性非肝硬化门静脉高压症的研究进展. 临床肝胆病杂志. 2017, 33: 348-353.
[14] 王曙照, 刘钊, 于红卫, 等. 特发性门脉高压症误诊为肝硬化临床分析.中国误诊学杂志, 2011, 11: 7065-7066.
[15] 王建军,荣义辉,靳雪源,等. 特发性门脉高压症11例临床特点分析. 人民军医, 2012, 55: 643-644.
[16] Guido M, Alves VAF, Balabaud C, et al. Histology of portal vascular changes associated with idiopathic non-cirrhotic portal hypertension: nomenclature and definition. Histopathology, 2019, 74: 219-226.
[17] Guido M, Sarcognato S, Sacchi D, et al. Pathology of idiopathic non-cirrhotic portal hypertension. Virchows Arch, 2018, 473: 23-31.