Bernard P H, Le Bail B, Cransac M, Barcina M G, Carles J, Balabaud C, Bioulac-Sage P
Unité de Transplantation Hépatique, Hôpital Pellegrin, Bordeaux, France.
J Hepatol. 1995 Apr;22(4):495-9. doi: 10.1016/0168-8278(95)80115-4.
We report the case of a 30-year-old male patient suffering from what was initially thought to be end-stage cryptogenic cirrhosis with portal hypertension and liver failure, who underwent liver transplantation. Histological examination of the surgical specimen showed incomplete septal cirrhosis. At the age of 17 this patient had presented pancytopenia and splenomegaly, which were treated by splenectomy. The surgeon discovered portal hypertension. Re-examination of the wedge liver biopsy taken at this time revealed features of idiopathic portal hypertension. This case clearly shows that incomplete septal cirrhosis may be a late manifestation of idiopathic portal hypertension. The presence of sinusoidal dilatation and peliosis as well as early evidence of fibrosis which are already visible on the initial biopsy and are still present on the late specimen, are indirect evidence of a continuous process which ultimately led to incomplete cirrhosis with liver failure.
我们报告了一例30岁男性患者的病例，该患者最初被认为患有终末期隐源性肝硬化伴门静脉高压和肝衰竭，并接受了肝移植。手术标本的组织学检查显示为不完全间隔性肝硬化。该患者17岁时出现全血细胞减少和脾肿大，接受了脾切除术治疗。外科医生发现了门静脉高压。此时对楔形肝活检进行复查，发现了特发性门静脉高压的特征。该病例清楚地表明，不完全间隔性肝硬化可能是特发性门静脉高压的晚期表现。最初活检时就已可见的窦性扩张、血囊肿以及纤维化的早期证据，在晚期标本中仍然存在，这些都是一个持续过程的间接证据，该过程最终导致了伴有肝衰竭的不完全肝硬化。
