Ashmila Hamza, Khatoon Nazia, Keaveny Andrew, Krishna Muli, Nakhleh Raouf
Laboratory Medicine and Pathology, Mayo Clinic in Florida, Jacksonville, FL, USA.
Department of Gastroenterology and Hepatology, Mayo Clinic in Florida, Jacksonville, FL, USA.
Int J Surg Pathol. 2025 May;33(3):571-576. doi: 10.1177/10668969241268406. Epub 2024 Aug 21.
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. Data was collected through retrospective patient medical records. 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. 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. 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.
可能导致门脉高压的原发性非纤维化疾病的鉴别诊断包括肝门脉硬化（HPS）、结节性再生性增生（NRH）和窦性阻塞综合征（SOS）。在本文中，我们描述了这些病变表现为门脉高压时的临床特征和预后。数据通过回顾性患者病历收集。近年来，患者（HPS：28例，NRH：17例，SOS：11例）的确诊更为频繁。所有组均出现门脉高压的体征和症状。所有患者都有复杂的病史。所有组均出现血清碱性磷酸酶升高，SOS组出现胆红素升高。HPS和NRH患者的肝脏成像提示肝硬化，而SOS患者未出现。HPS、NRH和SOS组分别有11%、12%和9%的患者接受了经颈静脉肝内门体分流术以处理门脉高压并发症，而分别有43%、24%和36%的患者接受了肝移植。HPS、NRH和SOS患者有复杂的病史，这可能促成了这些病变的发生。现在对它们的认识更为频繁。与HPS和NRH不同，SOS发生在肝移植受者中，与血清胆红素升高有关，且成像未提示存在晚期纤维化/肝硬化。肝移植似乎是治疗与HPS和NRH相关并发症的可行方法。SOS再次移植的结果不一。在评估原因不明的非纤维化门脉高压患者的肝脏标本时，应考虑HPS、SOS和NRH。原发性非纤维化性门脉高压的病因似乎在增加。鉴别诊断包括NRH、HPS和SOS。这些情况与复杂疾病相关，可能与治疗有关。当面对为评估门脉高压而进行的肝活检时，病理学家需要了解这种鉴别诊断。
