特发性门脉高压症的临床病理学特点

The clinical and pathological features of idiopathic portal hypertension

目的探讨特发性门脉高压症(idiopathic portal hypertension,IPH)的临床病理学特点。方法回顾性分析了9例IPH的临床及病理学资料,并对其肝脏标本进行常规病理学及免疫组化研究。结果9例IPH中,5例首发症状为上消化道出血和黑便,3例体检发现脾大脾亢而无临床症状,1例以血管瘤入院。入院检查脾肿大7例,胃底食道静脉曲张6例,腹水征4例,贫血者6例,肝功能正常或接近正常9例。病理组织学显示9例肝小叶结构基本正常,均未见假小叶形成及肝细胞坏死;9例均有不同程度汇管区纤维化,3例汇管区纤细的不全纤维间隔形成并向肝实质延伸,6例有门脉末支管壁纤维化;9例中有6例小叶内肝细胞有不同程度的水肿变性,5例肝窦有不同程度的扩张,2例肝窦扩张较明显,肝细胞萎缩,呈血管瘤样结构,2例有轻-中度肝腺胞3区大泡脂变。脾脏组织学符合淤血性脾肿大病理表现。结论IPH的临床表现与其他原因所致的肝硬化门脉高压相似,肝穿组织病理学可除外肝硬化,并有一定的特征。诊断时应与各种原因所致肝硬化门脉高压,肝窦阻塞综合征等相鉴别。

Objective To investigate the clinical and pathological features of idiopathic portal hypertension （IPH）. Methods The clinical manifestations and pathological changes of 9 IPH cases were analyzed by retrospective study. The pathology and immunohistochemistry were studied in these liver specimens. Results In all of the 9 IPH patients, 5 cases showed upper digestive tract hemorrhage and hematochezia as the first symptom ;3 cases presented splenomegaly without any symptom;1 case admitted as liver hemangioma. Seven cases were found splenomegaly by physical examination; 6 cases had oesophageal and gastric fundus varices;4 cases with ascite ;6 cases with anemia. Liver function test were normal or close to normal in all cases. Liver histology demonstrated lobular architecture maintained in all specimens. The liver had no cirrhosis or pseudonodule formation. Hepatocytic necrosis was absent in all specimens. A variable degree of portal fibrosis appeared in all specimens. Slender fibrous septa from the portal tracts and formation of introlobular slender fibrous septa were shown in 3 cases,fibrosis of intrahepatic terminal portal vein in 6 cases. Variable degree of hepatocyt- ic hydropic degeneration was shown in 6 of 9 cases. Five cases with variable degree of sinusoidal dilatation, massive si- nusoidal dilatation and parenchymal atrophy appeared as hemangioma-like structure in 2 cases. Mild to moderate degree of macrosteatosis located in hepatic acinus zone 3 were presented in 2 cases. Spleen histological examination showed the pathological change of congestive splenomegaly. Conclusion The clinical manifestation of IPH is as similar as that of cirrhosis with portal hypertension caused by other agents. Histopathological assessment of liver biopsy can exclude liver cirrhosis, which have some characteristics. The diagnosis of IPH needs to differentiate from cirrhosis with portal hypertension caused by other agents and hepatic sinusoidal obstruction syndrome.