PDF(Pubmed)
Abstract：
Primary myelofibrosis (PMF) is an infrequent etiology of noncirrhotic portal hypertension (PH). In clinical settings, non-cirrhotic PH is often misdiagnosed as cirrhotic PH. This case report details a patient who exhibited recurrent esophageal variceal hemorrhage and was initially misdiagnosed with cirrhosis. Initially poised for liver transplantation, the patient\'s liver biopsy revealed no significant cirrhosis but showed signs of extramedullary hematopoiesis (EMH). Following the accurate diagnosis of PMF, the patient underwent standard treatment, leading to an absence of recurrent gastrointestinal hemorrhage due to esophageal varices for nearly three years.
摘要：
原发性骨髓纤维化（PMF）是非肝硬化门脉高压（PH）的罕见病因。在临床环境中，非肝硬化PH常被误诊为肝硬化PH。该病例报告详细介绍了一名患者，该患者表现出复发性食管静脉曲张破裂出血，最初被误诊为肝硬化。最初准备进行肝移植，患者肝活检显示无明显肝硬化，但有髓外造血（EMH）征象。在对PMF进行准确诊断后，病人接受了标准治疗,导致因食管静脉曲张引起的胃肠道反复出血近3年。
