Department of Pathology, Sir Ganga Ram Hospital, New Delhi, India.
J Clin Pathol. 2011 Jul;64(7):592-8. doi: 10.1136/jcp.2010.087395.
A group of non-cirrhotic chronic liver diseases, all with sustained portal hypertension and clinically mistaken as cirrhosis, have been described under various names, apparently because of differences in pathological features. The pathogenesis is uncertain and they were believed to have a good prognosis until it was recently shown, from study of explant livers, that they had progressed to end stage disease, needing liver transplantation.
To describe detailed morphological features of such end stage non-cirrhotic disease and examine whether the diseases bearing various names are different or represent variable morphological expressions of one entity.
Morphological features of 10 native explant livers from patients with pre-transplant diagnosis of end stage cirrhosis but finally categorised as non-cirrhotic portal fibrosis were analysed along with the relevant clinical information.
Besides absence of criteria for cirrhosis, variable grades of obliterative changes in portal vein branches and portal fibrosis were consistently seen in all livers. Fibrous intimal thickening with luminal compromise in some medium and large sized portal veins was randomly distributed but appeared characteristic of this disease, very likely representing organised mural thrombi. Areas of closely placed nodular hyperplastic parenchyma separated by compressed hepatocytes, megasinusoids and peliotic changes were seen only in a proportion of cases.
Non-cirrhotic portal fibrosis is a justifiable name for this disease that can progress to end stage liver disease. It represents a single entity that has been considered as different diseases and given various names on the basis of the dominant element in its heterogeneous morphological manifestation.
一组非肝硬化的慢性肝病，均伴有持续的门静脉高压，并在临床上误诊为肝硬化，它们以不同的名称被描述，显然是由于病理特征的差异。其发病机制尚不清楚，并且由于研究表明它们已经进展为终末期疾病，需要进行肝移植，因此人们认为它们的预后良好。
描述这种终末期非肝硬化疾病的详细形态学特征，并检查具有不同名称的疾病是否不同，或者是否代表同一实体的不同形态学表现。
分析了 10 例来自于终末期肝硬化但最终归类为非肝硬化性门静脉纤维化的患者的移植前肝组织标本的形态学特征，并结合相关的临床资料进行分析。
除了缺乏肝硬化的标准外，所有肝脏均可见门静脉分支和门脉纤维化的程度不同的闭塞性改变。一些中大型门静脉的纤维性内膜增厚伴管腔狭窄，呈随机分布，但这种病变具有特征性，很可能代表已机化的壁内血栓。在部分病例中可见紧密排列的结节状增生性实质区，其间被受压的肝细胞、巨大窦和铁质沉着所分隔。
非肝硬化性门静脉纤维化是该疾病的合理命名，它可以进展为终末期肝病。它代表了一种单一实体，由于其异质性形态表现的主导因素，被认为是不同的疾病，并给予了不同的名称。
