目的:可能导致门脉高压的内在非纤维化疾病的鉴别诊断包括门静脉硬化(HPS),结节性再生增生(NRH),和正弦阻塞综合征(SOS)。在这篇文章中,我们描述了这些病变表现为门静脉高压时的临床特征和结局。方法:通过回顾性患者病历收集数据。结果:近年来发现患者(HPS:28,NRH:17,SOS:11)的频率更高。所有组均出现门静脉高压症的体征和症状。所有患者都有复杂的病史。所有组的血清碱性磷酸酶升高,SOS的胆红素升高。HPS和NRH的肝脏成像提示肝硬化,没有看到SOS。11%,12%,9%的HPS患者,NRH,和SOS分别,经颈静脉肝内门体分流术治疗门静脉高压症并发症,而43%,24%,和36%的患者,接受了肝脏移植.结论:HPS患者,NRH,SOS有复杂的病史,可能有助于这些病变的发展。他们现在更经常被认可。与HPS和NRH相比,SOS发生在肝移植受者中,与血清胆红素升高有关,和影像学没有提示晚期纤维化/肝硬化的存在。肝移植似乎是与HPS和NRH相关的并发症的可行治疗方法。SOS的重新移植产生了混合的结果。HPS,SOS,在评估无法解释的非纤维化门脉高压患者的肝标本时,应考虑NRH和NRH。关键信息:门脉高压的内在非纤维化原因似乎频率正在增加。鉴别诊断包括NRH,HPS,和SOS。这些疾病与复杂的疾病相关,可能是由于治疗。当进行肝活检以评估门静脉高压时,病理学家需要意识到这种鉴别诊断。
Aim: The differential diagnosis of intrinsic nonfibrotic conditions that may lead to portal hypertension include hepatoportal sclerosis (HPS), nodular regenerative hyperplasia (NRH), and sinusoidal obstruction syndrome (SOS). In this article, we characterize the clinical features and outcome of these lesions when they manifest as portal hypertension. Methods: Data was collected through retrospective patient medical records. Results: Patients (HPS: 28, NRH: 17, SOS: 11) were identified more frequently in recent years. All groups presented with signs and symptoms of portal hypertension. All patients had complex medical histories. An elevated serum alkaline phosphatase occurred in all groups and an elevated bilirubin with SOS. Imaging of the liver with HPS and NRH suggested cirrhosis, which was not seen with SOS. 11%, 12%, and 9% of patients in the HPS, NRH, and SOS respectively, underwent transjugular intrahepatic portosystemic shunt placement to manage the complications of portal hypertension, while 43%, 24%, and 36% of patients respectively, received a liver transplant. Conclusions: Patients with HPS, NRH, and SOS had complex medical histories, likely contributing to the development of these lesions. They are recognized more frequently now. In contrast to HPS and NRH, SOS occurred in liver transplant recipients, was associated with elevated serum bilirubin, and imaging did not suggest the presence of advanced fibrosis/cirrhosis. Liver transplantation appeared to be a viable treatment for complications related to HPS and NRH. Retransplantation for SOS yielded mixed results. HPS, SOS, and NRH should be considered when evaluating liver specimens from patients with unexplained nonfibrotic portal hypertension. Key message: Intrinsic nonfibrotic causes of portal hypertension appear to be increasing in frequency. The differential diagnosis includes NRH, HPS, and SOS. These conditions are associated with complex diseases and possibly due to treatments. Pathologists need to be aware of this differential diagnosis when presented with liver biopsies performed to assess portal hypertension.