Case report: POEMS syndrome with portal hypertension

被引:0
作者
Xu, Xiaotong [1 ,2 ]
Jing, Changyou [3 ]
Zhu, Tong [3 ]
Jiang, Minjie [1 ,2 ]
Fu, Yunlai [1 ,2 ]
Xie, Fang [1 ]
Li, Jianjun [3 ]
Meng, Qinghua [2 ]
机构
[1] Capital Med Univ, Beijing Youan Hosp, Beijing Inst Hepatol, Beijing, Peoples R China
[2] Capital Med Univ, Beijing Youan Hosp, Dept Oncol, Beijing, Peoples R China
[3] Capital Med Univ, Beijing Youan Hosp, Intervent Therapy Ctr Oncol, Beijing, Peoples R China
关键词
POEMS syndrome; portal hypertension; case report; ascites; hydrothorax; ASCITES; MANAGEMENT; SURVIVAL; SHUNT;
D O I
10.3389/fmed.2024.1373397
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
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.
引用
收藏
页数:8
相关论文
共 50 条
  • [1] POEMS syndrome with idiopathic portal hypertension: Autopsy case and review of the literature
    Inoue, Rie
    Nakazawa, Atsushi
    Tsukada, Nobuhiro
    Katoh, Yuko
    Nagao, Toshitaka
    Nakanuma, Yasuni
    Mukai, Kiyoshi
    PATHOLOGY INTERNATIONAL, 2010, 60 (04) : 316 - 320
  • [2] Reversal of pulmonary arterial hypertension in POEMS syndrome with thalidomide: a case report
    Tanaka, Toshikazu D.
    Misawa, Sonoko
    Yoshimura, Michihiro
    Kuwabara, Satoshi
    EUROPEAN HEART JOURNAL-CASE REPORTS, 2018, 2 (02)
  • [3] Portal hypertension and culture negative neutrocytic ascites in POEMS syndrome.
    Stepani, P
    Courouble, Y
    Postel, P
    Mezieres, P
    Tossou, H
    Couvelard, A
    Trophilme, D
    Barbare, JC
    Bories, C
    GASTROENTEROLOGIE CLINIQUE ET BIOLOGIQUE, 1998, 22 (12): : 1095 - 1097
  • [4] POEMS syndrome presents with a distended abdomen: A case report
    Wang, Wenshu
    Yang, Guibin
    JOURNAL OF INTERNATIONAL MEDICAL RESEARCH, 2023, 51 (01)
  • [5] POEMS syndrome and idiopathic portal hypertension: a possible association
    Campos, Sara
    Agostinho, Claudia
    Cipriano, Maria Augusta
    REVISTA ESPANOLA DE ENFERMEDADES DIGESTIVAS, 2017, 109 (05) : 393 - 393
  • [6] Coexistence of the POEMS syndrome with hepatitis B: a rare case report
    Celik, Yilmaz
    Aslan, Mehmet
    Sayin, Refah
    Cifci, Adem
    Canbaz, Esra Turan
    Kucukoglu, Mehmet Emin
    Dulger, Ahmet Cumhur
    WIENER KLINISCHE WOCHENSCHRIFT, 2016, 128 (3-4) : 150 - 152
  • [7] Case report: Primary myelofibrosis presenting with portal hypertension mimicking cirrhosis
    Gan, Xiayu
    Yu, Shengjie
    Zhu, Min
    Ning, Bo
    He, Song
    Xie, Xiaoyue
    Tu, Linshuang
    Yu, Huihong
    FRONTIERS IN MEDICINE, 2024, 11
  • [8] POEMS syndrome - a case report
    Ebhardt, H
    Eidner, T
    Berndt, A
    Katenkamp, D
    Kosmehl, H
    PATHOLOGE, 2000, 21 (03): : 255 - 259
  • [9] POEMS syndrome: A case report
    Oezden, Ali
    Sueleyman, Zeygam
    Seven, Guelseren
    Arat, Mutlu
    Akin, Alper
    Sener, Oezden
    Kuzu, Isinsu
    Keven, Kenan
    Ensari, Arzu
    Erden, Ayse
    Tan, Ersin
    TURKISH JOURNAL OF GASTROENTEROLOGY, 2008, 19 (04) : 276 - 280
  • [10] POEMS syndrome: a case report
    Mayayo Sinues, E.
    Martinez Garcia, R.
    Felipe Berlanga, F.
    Mazas Artasona, L.
    RADIOLOGIA, 2009, 51 (04): : 424 - 427