Ludwig J, Hashimoto E, Obata H, Baldus W P
Division of Medical Pathology, Mayo Clinic, Rochester, Minnesota.
Histopathology. 1993 Mar;22(3):227-34. doi: 10.1111/j.1365-2559.1993.tb00112.x.
Analysis of 25 liver biopsy specimens and one autopsy specimen from 26 Japanese patients (23 women and three men) with idiopathic portal hypertension revealed findings that collectively appeared diagnostic for the condition. Changes in the portal tract included capillary dilatation, phlebosclerosis, and fibro-elastosis of the stroma. Many portal veins were dilated and had herniated into the surrounding hepatic parenchyma. Portal vein obliteration and loss of bile ducts were a rare complication. The acinar architecture was disturbed by: 1) capillary and necro-inflammatory bridging, mostly between portal tracts and terminal hepatic veins; 2) the formation of isolated megasinusoids in a random distribution; 3) displaced and abnormally large hepatic vein branches with or without phlebosclerosis and 4) slender, curved fibrous septa (hairline septa). Early nodular regeneration was found in 25% of the cases. Our review supports the contention that incomplete septal cirrhosis may be a late manifestation of idiopathic portal hypertension. It is not clear whether the biopsy findings in Japanese patients differ only in severity from those in western patients, or whether the conditions differ pathogenetically. Some histopathological findings in the Japanese cases, in particular the necro-inflammatory changes, are difficult to reconcile with portal hypertension as a primary haemodynamic abnormality.
对26例日本特发性门静脉高压患者（23名女性和3名男性）的25份肝活检标本和1份尸检标本进行分析，发现了一些共同构成该病诊断依据的结果。门管区的变化包括毛细血管扩张、静脉硬化以及间质的纤维弹性组织增生。许多门静脉扩张并疝入周围肝实质。门静脉闭塞和胆管缺失是一种罕见的并发症。腺泡结构受到以下因素干扰：1）毛细血管和坏死性炎性桥接，主要发生在门管区和终末肝静脉之间；2）随机分布的孤立性大血窦形成；3）移位且异常粗大的肝静脉分支，伴有或不伴有静脉硬化；4）纤细、弯曲的纤维间隔（发丝样间隔）。25%的病例发现早期结节状再生。我们的综述支持不完全间隔性肝硬化可能是特发性门静脉高压晚期表现的观点。目前尚不清楚日本患者的活检结果与西方患者的活检结果仅仅是严重程度不同，还是在发病机制上存在差异。日本病例中的一些组织病理学发现，尤其是坏死性炎性改变，很难与作为主要血流动力学异常的门静脉高压相协调。
