Liew C T
Department of Pathology, University of Southern California, School of Medicine.
Changgeng Yi Xue Za Zhi. 1990 Sep;13(3):167-81.
Forty-two cases of hepatic venous outflow obstruction with biopsied and autopsied specimens were studied. The morphological changes in the liver and the value of liver tissue in the diagnosis among the different subgroups of this conditions were evaluated. Seven cases in the group with veno-occlusive disease (VOD) showed prominent changes in the sublobular veins and terminal hepatic venules and were characterized by acute disruption of the vessel walls. Two cases showed thrombosis in which there were history of chemotherapy. In the group with Budd-Chiari's syndrome (BCS), in addition to the involvement of the large veins, thrombosis was observed in the small hepatic vein radicles in five out of six cases with myeloproliferative related disorders. Thrombosis was rarely present in cases associated with idiopathic and web lesions in BCS but frequent large vessel tumor thrombosis was noted in the group with malignant tumors. The hepatic parenchymal changes in the VOD group and the tumor related cases in BCS group were acute perivenular congestion, hemorrhage and coagulative necrosis, whereas the histological changes in other subgroups of BCS were perivenular scarring, dilatation of the sinusoids and rarely, reversed lobulation. Subtle differences were noted in the vascular system and the liver parenchyma between each subgroup. If good clinical information is available, it is possible to differentiate the various etiologies in hepatic venous outflow obstruction in the liver by morphology.
对42例有活检和尸检标本的肝静脉流出道梗阻病例进行了研究。评估了肝脏的形态学变化以及该疾病不同亚组中肝组织在诊断中的价值。静脉闭塞性疾病（VOD）组的7例患者小叶下静脉和终末肝小静脉有明显变化，其特征是血管壁急性破坏。2例有化疗史的患者出现血栓形成。在布加综合征（BCS）组中，除大静脉受累外，6例骨髓增殖性相关疾病患者中有5例肝小静脉分支出现血栓形成。BCS中与特发性和网状病变相关的病例很少出现血栓形成，但恶性肿瘤组中频繁出现大血管肿瘤血栓形成。VOD组和BCS组肿瘤相关病例的肝实质变化为急性静脉周围充血、出血和凝固性坏死，而BCS其他亚组的组织学变化为静脉周围瘢痕形成、肝血窦扩张，很少出现肝小叶反转。各亚组之间在血管系统和肝实质方面存在细微差异。如果有良好的临床信息，通过形态学有可能区分肝脏中肝静脉流出道梗阻的各种病因。
