多发性神经病,器官肿大,内分泌病,M-蛋白,皮肤改变(POEMS)综合征是一种罕见的浆细胞异常疾病,涉及多个器官并伴有特发性非肝硬化门脉高压。在英国文学中仅有四次报道。这里,我们介绍了第一例62岁男性POEMS综合征患者经经颈静脉肝内门体分流术(TIPS)治疗的门静脉高压症,在他有10天的黑便病史之后.由于患者表现为多发性神经病,因此诊断为POEMS综合征,单克隆浆细胞增殖性疾病,硬化骨病变,脾肿大,淋巴结病,腹水,甲状腺功能减退,和色素沉着过度。食管静脉曲张证实了门静脉高压的存在,充血和水肿的胃体,脾肿大,以及腹水液的血清-腹水白蛋白梯度超过11g/L(被认为与POEMS综合征相关的浓度)的腹水,因为没有发现其他原因。患者入院时禁食并接受保守药物治疗,但是黑便的症状很快在恢复饮食后的1周内复发。在进行TIPS和静脉栓塞后,出血症状得到有效控制,患者随后出现肝性脑病,最终导致死亡。POEMS综合征合并特发性非肝硬化门静脉高压症的消化道出血提示预后不良。鉴于这是第一位接受TIPS的患者,尽管肝性脑病的发病率有所增加,TIPS仍可用于难治性静脉曲张破裂出血。
Polyneuropathy, organomegaly, endocrinopathy, M-protein, skin changes (POEMS) syndrome is a rare plasma cell dyscrasia disease involving multiple organs combined with idiopathic non-cirrhotic portal hypertension. It has been reported only four times in the English literature. Here, we present the first case of a 62-year-old male POEMS syndrome patient with portal hypertension treated with the transjugular intrahepatic portosystemic shunt (TIPS), after he presented with a 10-day history of
melena. The diagnosis of POEMS syndrome was given because the patient presented with polyneuropathy, monoclonal plasma cell proliferative disorder, sclerotic bone lesions, splenomegaly, lymphadenopathy, ascites, hypothyroidism, and hyperpigmentation. The presence of portal hypertension was confirmed by esophageal varices, congested and edematous stomach body, splenomegaly, and transudate ascites in which the serum-ascites albumin gradient of ascites fluid was over 11 g/L (a concentration considered to be associated with POEMS syndrome), as no other causes were found. The patient fasted and received conservative drug treatments on admission, but symptoms of
melena soon recurred within 1 week after resuming his diet. After TIPS and venous embolization were performed, symptoms of bleeding were effectively controlled, while the patient subsequently developed hepatic encephalopathy, which ultimately led to death. The presence of gastrointestinal bleeding in POEMS syndrome with idiopathic non-cirrhotic portal hypertension indicates a poor prognosis. Given that this was the first patient to receive TIPS, and although the incidence of hepatic encephalopathy has increased, TIPS is still acceptable for refractory variceal bleeding.