肝静脉闭塞型Budd-Chiari氏综合征患者病灶组织细胞凋亡及相关基因表达的观察

目的从肝静脉内膜增生、细胞凋亡及其相关基因角度,探讨肝静脉阻塞型布-加氏综合征的发病机理。方法选择32例肝静脉阻塞型Budd-Chiari氏综合征患者手术中切下的标本,即闭塞肝静脉以及增生狭窄的病灶组织,利用光镜、电镜进行病理学检查,同时进行免疫组织化学检查,观察病变组织的组织学变化、凋亡细胞及其相关基因表达情况;并与正常肝静脉和下腔静脉对照。结果内膜组织不规则增生,弹力纤维不完整,内见散在平滑肌细胞及大量纤维结缔组织增生,部分透明样变性或玻璃样变性,伴有大量炎性细胞浸润。病灶组织异常增生和凋亡并存,凋亡细胞数和PCNA、bcl-2阳性表达明显高于对照组。结论肝静脉病变组织不规则异常增生、细胞凋亡异常可能是肝静脉阻塞型布-加氏综合征的发病机理之一或疾病过程中的基本病理表现之一。

Apoptosis of pathological tissues and expression of apoptosis-related genes in hepatic venous stricture Budd-Chiari syndrome

OBJECTIVE: To investigate the pathogenesis of hepatic venous stricture Budd-Chiari syndrome from the point of view of hepatic venous intimal proliferation, apoptosis and apoptosis-related genes. METHODS: Thirty samples resected from patients with hepatic venous stricture Budd-Chiari syndrome and 21 samples of normal hepatic vein and vena cava obtained from necropsy of fresh corps as controls were examined. The paraffin sections of the samples were stained with hematoxyline and eosin and diaminobenzidine respectively and observed under light microscope to examine the pathology and calculate the apoptotic cells. SP immunohistochemistry was used to detect the expression of Bcl-2 and Bax. Proliferating cell nuclear antigen (PCNA) expression was detected by SABC immunohistochemistry. RESULTS: Light microscopy showed irregular proliferation in the tunica intima and tunica media, especially in the former. Incomplete elastic fiber, spotted smooth muscle cells, proliferation of fibrous connective tissue, and degeneration of partial tissues, and infiltration of inflammatory cells, etc. were observed. In the endothelial tissue TUNEL positive cells was 1.94 +/- 0.64 in the Budd-Chiari syndrome patients, significantly higher than that in the controls (0.56 +/- 0.21, P < 0.002); and PCNA positive cells was 3.46 +/- 0.51 in the Budd-Chiari syndrome patients, significantly higher than that in the controls (0.78 +/- 0.16, P < 0.0005). The bcl-2 expression rate was 8.56 +/- 1.17 in the Budd-Chiari syndrome patients, significantly higher than that in the normal controls (4.34 +/- 1.28, P < 0.0005); Bax expression was 3.95 +/- 1.31 in the Budd-Chiari syndrome patients, significantly lower than that in the normal controls (5.06 +/- 1.21, P < 0.001); and the bcl/Bax ratio of the cells in the tunicae intima and media of the normal hepatic veins was 0.914 +/- 0.334, significantly lower than that in the Budd-Chiari syndrome patients (2.402 +/- 1.021, P < 0.01). Electron microscopy showed that in comparison with the normal tissues the pathological tissues had more synthesized smooth muscle cells and lacked normal myoendothelial structure. CONCLUSION: Irregular proliferation of tunica intima of hepatic vein and apoptosis may be one of the mechanisms of hepatic venous stricture Budd-Chiari syndrome.