Gan Xiayu, Yu Shengjie, Zhu Min, Ning Bo, He Song, Xie Xiaoyue, Tu Linshuang, Yu Huihong
Department of Gastroenterology, The Second Affiliated Hospital of Chongqing Medical University, Chongqing, China.
Department of Urology, The Second Affiliated Hospital of Chongqing Medical University, Chongqing, China.
Front Med (Lausanne). 2024 May 3;11:1375571. doi: 10.3389/fmed.2024.1375571. eCollection 2024.
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.
原发性骨髓纤维化（PMF）是非肝硬化门静脉高压（PH）的一种罕见病因。在临床环境中，非肝硬化性PH常被误诊为肝硬化性PH。本病例报告详细介绍了一名出现复发性食管静脉曲张出血且最初被误诊为肝硬化的患者。该患者最初准备接受肝移植，但其肝活检显示无明显肝硬化，但有髓外造血（EMH）迹象。在准确诊断为PMF后，患者接受了标准治疗，近三年来未再因食管静脉曲张出现复发性胃肠道出血。
