肝脏 ›› 2020, Vol. 25 ›› Issue (8): 860-862.

• 其他肝病 • 上一篇    下一篇

POEMS综合征误诊临床分析

李璐, 杨仙珊, 孔明, 郑素军, 陈煜   

  1. 100069 首都医科大学附属北京佑安医院疑难肝病及人工肝中心,肝衰竭与人工肝治疗研究北京市重点实验室
  • 收稿日期:2020-03-24 出版日期:2020-08-31 发布日期:2020-09-04
  • 通讯作者: 陈煜,Email:chybeyond@163.com
  • 基金资助:
    国家重点研发计划资助项目(2017YFA0103000);国家科技重大专项“艾滋病和病毒性肝炎等重大传染病防治”(2017ZX10203201-005);北京市医院管理局临床医学发展专项经费资助(ZYLX201806);白求恩医学科学研究基金资助项目

Clinical analysis of 4 cases of POEMS syndrome and literature review

LI Lu1,2, YANG Xian-shan1,2, KONG Ming1,2, ZHENG Su-jun1,2, CHEN Yu1,2*   

  1. 1. Difficult & complicated liver diseases and artificial liver center, Beijing You An Hospital, Capital Medical University, Beijing 100069, China;
    2. Beijing Municipal Key Laboratory of Liver Failure and Artificial Liver Treatment Research, Beijing 100069, China
  • Received:2020-03-24 Online:2020-08-31 Published:2020-09-04
  • Contact: CHEN Yu, Email: chybeyond@163.com

摘要: 目的 探讨POEMS综合征的发病特点及临床表现。方法 2017年1月至2019年8月就诊于北京佑安医院的4例患者,根据就诊原因、临床表现,检查结果,对上述患者进行相关误诊分析。结果 1例患者以周围神经为首发症状,其余3例因腹腔积液、肝脾肿大就诊,免疫球蛋白A单株峰y轻链型3例,免疫球蛋白G单株峰y轻链型1例,4例VEGF均大于800 pg/mL,病理无肝纤维化表现。结论 POEMS综合征是多系统的病变,其发病率低,误诊率高。肝、脾肿大合并腹腔积液可能是部分POEMS综合征患者的首发症状。

关键词: POEMS综合征, 腹腔积液, 器官肿大, M蛋白, 肝组织病理

Abstract: Objective To improve the understanding of POEMS syndrome and reduce the incidence of misdiagnosis. Methods 4 patients who were treated in our department from January 2017 to August 2019 were collected. According to the causes of the disease, clinical features and examination results, relevant misdiagnosis analysis and literature review of the above patients were conducted to summarize their characteristics. Results 1 case had peripheral nerve and other 3 cases had ascites or hepatosplenomegaly as the first symptom on admission. 3 cases were Ig A lamda positive, one was IgG lamda positive. The VEGF levels of them were higher than 800 pg/ml, and no fibrosis in liver tissue pathology. Conclusion POEMS syndrome is a multi-systemic disease with low incidence and high misdiagnosis rate. Hepatomegaly and splenomegaly with peritoneal effusion may be the first symptom of some patients with POEMS syndrome. Clinicians should pay attention to multi-system inspection to reduce misdiagnosis and improve clinical diagnosis rate.

Key words: POEMS syndrome, Peritoneal effusion, Organ enlargement, M protein, Liver tissue pathology