Jacobi David, de Muret Anne, Arbeille Brigitte, Perarnau Jean-Marc
Department of Hepatogastroenterology, Centre Hospitalier Universitaire Trousseau, avenue de la République, 37044 Tours Cedex 9, France.
Eur J Gastroenterol Hepatol. 2006 May;18(5):549-51. doi: 10.1097/00042737-200605000-00017.
Non-cirrhotic perisinusoidal hepatic fibrosis is a process of imprecise pathogenesis involving collagenization of the space of Disse. Exposure to chemicals, auto-immunity, thrombophilia and/or infections are suspected primary agents. Here, we present the case of a patient who developed severe portal hypertension with histological features suggesting a non-cirrhotic perisinusoidal hepatic fibrosis. A 52-year-old man was hospitalized for oesophageal variceal haemorrhage. Liver cirrhosis or portal vein thrombosis were absent as attested by laboratory tests, duplex sonography, computed tomography scan and histological examination of a liver biopsy specimen. Presinusoidal portal hypertension was suggested by a normal wedge-free hepatic vein gradient. Only electron microscopy examination of a liver biopsy specimen could disclose perisinusoidal fibrosis. This was most probably secondary to a combined chemotherapy received 4 years earlier for non-Hodgkin large-cell lymphoma. As variceal ligation failed to control oesophageal varices while liver function tests were normal, a transjugular intrahepatic portosystemic shunt (TIPS) was performed. This dramatically improved the signs of portal hypertension. This case illustrates the use of TIPS in the treatment of portal hypertension secondary to non-cirrhotic perisinusoidal fibrosis.
非肝硬化性肝窦周纤维化是一种发病机制尚不明确的过程，涉及狄氏间隙的胶原化。化学物质暴露、自身免疫、血栓形成倾向和/或感染被怀疑是主要病因。在此，我们报告一例患者，其出现严重门静脉高压，组织学特征提示为非肝硬化性肝窦周纤维化。一名52岁男性因食管静脉曲张出血入院。实验室检查、双功超声、计算机断层扫描和肝活检标本的组织学检查均证实无肝硬化或门静脉血栓形成。正常的无肝静脉楔压梯度提示肝窦前门静脉高压。只有肝活检标本的电子显微镜检查才能发现肝窦周纤维化。这很可能继发于4年前因非霍奇金大细胞淋巴瘤接受的联合化疗。由于肝功能检查正常时曲张静脉结扎未能控制食管静脉曲张，遂进行了经颈静脉肝内门体分流术（TIPS）。这显著改善了门静脉高压的体征。该病例说明了TIPS在治疗非肝硬化性肝窦周纤维化继发的门静脉高压中的应用。
