特发性非肝硬化性门静脉高压症一例并文献复习

Idiopathic non-cirrhotic portal hypertension:a case report and literature review

患者男,因间断腹胀1个月余,于2022年3月1日到我院就诊。实验室检查提示患者有脾功能亢进、高甘油三酯血症。腹部增强CT提示脾脏肿大、门静脉高压。胃镜检查提示食管胃底静脉曲张、反流性食管炎(LA-A级)、慢性非萎缩性胃炎伴糜烂。肝组织穿刺活检病理诊断为肝门静脉纤维化。免疫组织化学可见汇管区周围片状和带状肝窦内皮CD34(+),提示动脉供血优势。铁染色:大部分肝细胞质阳性,窦细胞阳性(考虑继发性),未见肝硬化。结合临床表现综合诊断为特发性非肝硬化性门静脉高压症。给予患者口服卡维地洛治疗后腹胀症状好转,出院。由于特发性非肝硬化性门静脉高压症较为罕见,早期症状隐匿,需要联合多种检查手段才能确诊。因此,笔者报道本例的诊疗过程,希望对临床医师提供参考。

A male patient presented to our hospital on March 1,2022,with intermittent abdominal distension for over a month.Laboratory tests revealed splenic hypersplenism and hypertriglyceridemia.Abdominal enhanced CT indicated splenomegaly and portal hypertension.Gastroscopy revealed esophagogastric varices,reflux esophagitis(LA grade A),and chronic non-atrophic gastritis with erosion.Liver biopsy pathology showed portal vein fibrosis.Immunohistochemistry showed CD34(+)endothelial cells around the central vein,suggesting arterial blood supply dominance.Iron staining was positive in most hepatocytes and sinusoidal cells(consider secondary causes),without evidence of cirrhosis.Combining clinical manifestations,the diagnosis was idiopathic non-cirrhotic portal hypertension.The patient's abdominal distension improved with oral carvedilol treatment,and he was discharged.As idiopathic non-cirrhotic portal hypertension is rare with subtleearly symptoms,a combination of various diagnostic methods is necessary for confirmation.Therefore,this report aims to provide clinical reference for physicians by documenting the diagnostic and therapeutic process of this case.