Kakar Sanjay, Batts Kenneth P, Poterucha John J, Burgart Lawrence J
Department of Pathology, Veteran Affairs and University of California Medical Center, San Francisco, CA, USA.
Mod Pathol. 2004 Jul;17(7):874-8. doi: 10.1038/modpathol.3800073.
Impairment of venous outflow from the liver manifests as zone 3 sinusoidal dilatation and congestion (SDC) in liver biopsy. The spectrum of histologic changes in portal tracts has not been described. We studied liver biopsies from 34 patients with a confirmed diagnosis of venous outflow impairment (VOI). Liver transplant recipients and biopsies with cirrhosis and hepatic neoplasms were excluded. Clinical records were reviewed for laboratory tests and radiographic findings. In all, 19 patients had right heart disease, 13 had classic Budd-Chiari syndrome and two had veno-occlusive disease. Liver chemistry tests showed elevated liver transaminases (n=21; 61.8%), elevated alkaline phosphatase (n=31; 91.2%) and GGT (all 13 cases tested). The elevation in ALT and AST was mild (below 200 U/l in all cases), while alkaline phosphatase (ALP) was elevated above 500 U/l in nine (26.5%) patients and above 1000 U/l in three cases. On biopsy, all cases showed SDC. The portal tracts showed (a) portal expansion with bile ductular proliferation (n=16; 47.1%) accompanied by lymphoplasmacytic infiltrate (n=10), lymphocytic cholangitis (n=3) and portal or periportal fibrosis (n=11), (b) Portal and/or periportal fibrosis without ductular proliferation (n=3; 8.8%) or (c) Normal portal tracts (n=15; 44.1%). The combination of elevated ALP and bile ductular changes on biopsy suggested chronic bile duct disease. Ultrasound/CT scan evaluation of bile ducts in 26 patients showed no biliary tree abnormality. Antimitochondrial antibody testing in eight cases also yielded negative results. In conclusion, bile ductular proliferation, portal inflammation and portal-based fibrosis are commonly seen in liver biopsies of patients with VOI even in the absence of bile duct disease. These changes are often accompanied by elevated ALP and GGT and can lead to the suspicion of chronic biliary disease. In the absence of demonstrable abnormalities in the biliary tree, these changes can be attributed to venous outflow impairment.
肝脏静脉流出道受损在肝活检中表现为3区肝血窦扩张和淤血(SDC)。门静脉区域的组织学变化谱尚未见描述。我们研究了34例确诊为静脉流出道受损(VOI)患者的肝活检标本。排除肝移植受者以及伴有肝硬化和肝肿瘤的活检标本。查阅临床记录以获取实验室检查和影像学检查结果。其中,19例患者患有右心疾病,13例患有典型布加综合征,2例患有肝小静脉闭塞病。肝脏生化检查显示肝转氨酶升高(n = 21;61.8%)、碱性磷酸酶升高(n = 31;91.2%)以及γ-谷氨酰转肽酶升高(所有13例检测病例)。谷丙转氨酶(ALT)和谷草转氨酶(AST)升高程度较轻(所有病例均低于200 U/L),而9例(26.5%)患者碱性磷酸酶(ALP)升高超过500 U/L,3例超过1000 U/L。活检时,所有病例均显示肝血窦扩张。门静脉区域表现为:(a)门静脉扩张伴胆小管增生(n = 16;47.1%),伴有淋巴浆细胞浸润(n = 10)、淋巴细胞性胆管炎(n = 3)和门静脉或门周纤维化(n = 11);(b)门静脉和/或门周纤维化但无胆小管增生(n = 3;8.8%);或(c)正常门静脉区域(n = 15;44.1%)。活检时碱性磷酸酶升高与胆小管变化相结合提示慢性胆管疾病。26例患者的胆管超声/CT扫描评估未显示胆管树异常。8例患者的抗线粒体抗体检测结果也为阴性。总之,即使在无胆管疾病的情况下,VOI患者的肝活检中常见胆小管增生、门静脉炎症和门周纤维化。这些变化常伴有碱性磷酸酶和γ-谷氨酰转肽酶升高,并可能导致怀疑慢性胆道疾病。在胆管树未发现明显异常的情况下,这些变化可归因于静脉流出道受损。