关键词: 特发性, 非硬化性门静脉高压症, 临床, 病理, 特征

Abstract: Objective Analysis of clinicopathologic features in patients with idiopathic non-cirrhotic portal hypertension. Methods 30 cases of patients admitted to our hospital and diagnosed as idiopathic non-cirrhotic portal hypertension by pathology between February 2019 and February 2022 were selected as the observation group, with 11 males and 19 females, and the average age was (59.5±4.1) years old, and another 30 cases of patients diagnosed as hepatitis B-related cirrhotic portal hypertension by physical examination during the same period were selected as the control group, with 20 males and 10 females. The average age was (59.4±4.2) years old, comparing the clinical features of the two groups, the pathological features of the observation group and the clinical scores of the two groups. Results In the observation group, the percentage of females, the percentage of patients whose first symptom was gastrointestinal bleeding, abdominal discomfort, splenomegaly on CT examination, complications of upper gastrointestinal bleeding, and esophageal varices were 63.3%, 30%, 26.7%, 30%, 33.3%, and 20%, which were higher than those in the control group [33.3%, 6.7%, 6.7%, 6.7%, 10%, and 3.3%], respectively; and the percentage of hepatic The percentage of splenomegaly, alanine aminotransferase, aspartate aminotransferase, total bilirubin, creatinine level, platelet count, leukocyte count, neutrophil count, and prothrombin time were 13.3%, (41.5±5.6) U/L, (46.2±8.3) U/L, (19.25±2.4) μmol/L, (122.3±12.3) μmol/L, respectively, (57.6±21.6) × 10⁹/L, (2.6±1.7) × 10⁹/L, (22.8±4.7) × 10⁹/L, and (15.3±2.5) s, respectively, which were lower than those in the control group [46.7%, (72.3±6.8) U/L, (75.9±5.6) U/L, (32.6±8.5) μmol/L, (152.3± 25.6) μmol/L, (65.3±31.2) × 10⁹/L, (5.6±2.1) × 10⁹/L, (36.5±8.9) × 10⁹/L, and (18.2±3.1) s], and D-dimer, prothrombin activity, and glomerular filtration rate were (0.9±0.4), (71.2±3.2)%, (109.8± 23.2) mL·min^-1, which were higher than those in the control group [(0.5±0.1), (62.3±2.5)%, (101.2±18.6) mL·min^-1], respectively, and the differences were statistically significant (P<0.05). The main changes of observation group were stromal fibrosis in the portal tract, fibrous occlusion of the small branches of the portal vein, and dilatation and thickening of the small branches of the portal vein at the end of the confluent area. The hepatic sinusoids adjacent to the hepatic parenchyma are dilated to varying degrees, and the walls of the larger portal veins are thickened, in which smooth muscle hyperplasia is accompanied by fibrosis, and even portal vein sclerosis with thrombosis occurs. Some patients have prolonged disease, incomplete fibrous septum, and even nodular regenerative hyperplasia. The Child-Pugh score, Model for End-Stage Liver Disease (MELD) score, APRI score, and liver stiffness of the observation group were (6.1±0.8), (10.1±0.5), (0.7±0.2), (0.8±0.2) kPa, which were lower than those of the control group [(8.2±0.9), (16.3±2.5), respectively, (2.5±0.3) points, (12.6±0.5) kPa], and the percentage of positive CD34 immunostaining was 90%, which was higher than 40% in the control group, and the difference was statistically significant (P<0.05). Conclusion Idiopathic non-cirrhotic portal hypertension has a complex etiology and is relatively rare clinically, with a high percentage of complications related to portal hypertension, especially gastrointestinal bleeding. In patients with severe portal hypertension but mild hepatic impairment, good hepatic reserve function, and low liver stiffness, the diagnosis of idiopathic non-cirrhotic portal hypertension should be strongly suspected and made in conjunction with clinical, laboratory, imaging, and pathological manifestations.

Key words: Idiopathic, Non-sclerotic portal hypertension, Clinical, Pathological, Features