Xu Xiaotong, Jing Changyou, Zhu Tong, Jiang Minjie, Fu Yunlai, Xie Fang, Li Jianjun, Meng Qinghua
Beijing Institute of Hepatology, Beijing Youan Hospital, Capital Medical University, Beijing, China.
Department of Oncology, Beijing Youan Hospital, Capital Medical University, Beijing, China.
Front Med (Lausanne). 2024 Jul 23;11:1373397. doi: 10.3389/fmed.2024.1373397. eCollection 2024.
This patient was an elderly patient with abdominal distension and shortness of breath. According to relevant examinations, his condition was initially considered to be related to cirrhosis, but pathological biopsy confirmed the diagnosis of noncirrhotic portal hypertension of unknown etiology. The portal vein pressure was significantly reduced after transjugular intrahepatic portosystemic shunt (TIPS). Nevertheless, the relief of the hydrothorax and ascites was not significant, and the numbness in both lower limbs gradually worsened. POEMS syndrome was ultimately diagnosed following a comprehensive examination. After two courses of bortezomib combined with dexamethasone, the patient died due to a systemic infection. The clinical symptoms of the patient were atypical, as was the presence of portal hypertension, which hindered the diagnosis of POEMS. Due to the patient's advanced age, the diagnosis was delayed, and the prognosis was poor. This case reminds clinicians that POEMS patients can also have portal hypertension as the main manifestation.
该患者为老年患者，有腹胀和呼吸急促症状。根据相关检查，其病情最初被认为与肝硬化有关，但病理活检确诊为病因不明的非肝硬化门静脉高压症。经颈静脉肝内门体分流术（TIPS）后门静脉压力显著降低。然而，胸腔积液和腹水的缓解并不明显，且双下肢麻木逐渐加重。综合检查后最终诊断为POEMS综合征。在接受两疗程硼替佐米联合地塞米松治疗后，患者因全身感染死亡。该患者的临床症状不典型，门静脉高压的表现也不典型，这给POEMS的诊断带来了困难。由于患者年龄较大，诊断延迟，预后较差。该病例提醒临床医生，POEMS患者也可能以门静脉高压为主要表现。
