以腹胀、双下肢水肿、气促为表现的POEMS综合征1例及文献复习（英文）

POEMS syndrome presenting with abdominal distension, lower limb edema and shortness of breath: A case report and literature review

POEMS综合征是一种少见的副肿瘤性疾病。中南大学湘雅医院消化内科收治1例60岁女性POEMS综合征患者,其腹胀病史超过1年,并伴有严重的双下肢水肿及气促;检查结果提示多发性周围神经病变、M蛋白血症、脾肿大、淋巴结肿大、甲状腺功能减退、血管外容量负荷超载、硬化性骨病变、VEGF升高和肺动脉高压。根据POEMS综合征的最新诊断标准,患者满足2个强制性主要标准、2个其他主要标准和3个次要标准,排除鉴别诊断后POEMS综合征诊断明确。患者接受地塞米松及来那度胺治疗,临床症状得到明显改善。POEMS综合征的发病机制尚不完全清楚,VEGF水平的升高可能与上述临床表现有关。该患者病史超过14个月,严重影响其生活质量,临床上应提高对POEMS综合征早期诊断和早期治疗的意识。

POEMS syndrome is a rare paraneoplastic disorder. A 60-year-old female patient was admi t ted to the Department of Gastroenterology, Xiangya Hospital of Central South University (Changsha, China), complaining of abdominal distension, severe edema of both lower limbs and shortness of breath for more than 1 year. A f ter intensive and careful medical investigations, the patient manifested with polyneuropathy, M-proteinemia, splenomegaly, lymphadenopathy, hypothyroidism, extravascular volume overload, sclerotic bone lesions, elevated VEGF and pulmonary hypertension. According to the latest diagnostic criteria of POEMS syndrome, this patient met two mandatory major criteria, two other major criteria and three minor criteria, the diagnosis was clear a f ter ruling out differential diagnosis. The patient was treated with dexamethasone and lenalidomide, which relieved her clinical symptoms. The pathogenesis of POEMS syndrome is not fully understood;however, increased levels of vascular endothelial growth factor may contribute to most of the clinical manifestations. This patient had been in physical discomfort for more than 14 months, which seriously a f fected her quality of life. Clinically, the awareness of early diagnosis and treatment of POEMS syndrome should be improved.