{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,26]],"date-time":"2026-08-26T20:21:10Z","timestamp":1787775670413,"version":"build-2784847793"},"reference-count":18,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"11","content-domain":{"domain":["lww.com","ovid.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2022,11]]},"abstract":"<jats:sec>\n                    <jats:title\/>\n                    <jats:p>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.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.4103\/njcp.njcp_360_22","type":"journal-article","created":{"date-parts":[[2022,11,23]],"date-time":"2022-11-23T01:26:28Z","timestamp":1669166788000},"page":"1939-1944","update-policy":"https:\/\/doi.org\/10.1097\/lww.0000000000001000","source":"Crossref","is-referenced-by-count":7,"title":["A POEMS syndrome patient with idiopathic non-cirrhotic portal hypertension received the transjugular intrahepatic portosystemic shunt"],"prefix":"10.4103","volume":"25","author":[{"given":"Y","family":"Chen","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J","family":"Lin","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"X","family":"Jiang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Q","family":"Zhou","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"H","family":"Zhang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"276","reference":[{"key":"R1-24-20240907","doi-asserted-by":"crossref","first-page":"311","DOI":"10.1097\/00005792-198007000-00006","article-title":"Plasma cell dyscrasia with polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes: The POEMS syndrome. Report on two cases and a review of the literature","volume":"59","author":"Bardwick","year":"1980","journal-title":"Medicine (Baltimore"},{"key":"R2-24-20240907","doi-asserted-by":"crossref","first-page":"872","DOI":"10.1002\/ajh.26240","article-title":"POEMS syndrome: 2021 Update on diagnosis, risk-stratification, and management","volume":"96","author":"Dispenzieri","year":"2021","journal-title":"Am J Hematol"},{"key":"R3-24-20240907","doi-asserted-by":"crossref","first-page":"e975","DOI":"10.1212\/WNL.0000000000008062","article-title":"Prevalence, clinical profiles, and prognosis of POEMS syndrome in Japanese nationwide survey","volume":"93","author":"Suichi","year":"2019","journal-title":"Neurology"},{"key":"R4-24-20240907","doi-asserted-by":"crossref","first-page":"1428","DOI":"10.3389\/fimmu.2019.01428","article-title":"Characteristics of 1946 cases of POEMS syndrome in Chinese subjects: A literature-based study","volume":"10","author":"Wang","year":"2019","journal-title":"Front Immunol"},{"key":"R5-24-20240907","doi-asserted-by":"crossref","first-page":"1661","DOI":"10.1007\/s00277-013-1829-7","article-title":"The characteristics of ascites in patients with POEMS syndrome","volume":"92","author":"Cui","year":"2013","journal-title":"Ann Hematol"},{"key":"R6-24-20240907","doi-asserted-by":"crossref","first-page":"819","DOI":"10.1007\/s00277-010-1149-0","article-title":"Clinical characteristics and long-term outcome of patients with POEMS syndrome in China","volume":"90","author":"Li","year":"2011","journal-title":"Ann Hematol"},{"key":"R7-24-20240907","doi-asserted-by":"crossref","first-page":"e97","DOI":"10.14309\/crj.2017.97","article-title":"Pseudo Symmer's pipe-stem fibrosis in idiopathic noncirrhotic portal hypertension associated with POEMS syndrome","volume":"4","author":"Abby Philips","year":"2017","journal-title":"ACG Case Rep J"},{"key":"R8-24-20240907","doi-asserted-by":"crossref","first-page":"393","DOI":"10.17235\/reed.2017.4623\/2016","article-title":"POEMS syndrome and idiopathic portal hypertension: A possible association. Revista espanola de enfermedades digestivas: Organo oficial de la Sociedad Espanola de Patologia Digestiva","volume":"109","author":"Campos","year":"2017","journal-title":"Rev Esp Enferm Dig"},{"key":"R9-24-20240907","doi-asserted-by":"crossref","first-page":"316","DOI":"10.1111\/j.1440-1827.2009.02513.x","article-title":"POEMS syndrome with idiopathic portal hypertension: Autopsy case and review of the literature","volume":"60","author":"Inoue","year":"2010","journal-title":"Pathol Int"},{"key":"R10-24-20240907","doi-asserted-by":"crossref","first-page":"9","DOI":"10.1186\/s12878-017-0078-8","article-title":"Portal hypertension as the initial manifestation of POEMS syndrome: A case report","volume":"17","author":"Wu","year":"2017","journal-title":"BMC Hematol"},{"key":"R11-24-20240907","doi-asserted-by":"crossref","first-page":"e268","DOI":"10.1212\/WNL.0000000000009940","article-title":"Clinical characteristics, risk factors, and outcomes of POEMS syndrome: A longitudinal cohort study","volume":"95","author":"Keddie","year":"2020","journal-title":"Neurology"},{"key":"R12-24-20240907","doi-asserted-by":"crossref","first-page":"5246","DOI":"10.3748\/wjg.v23.i28.5246","article-title":"Refractory hepatic encephalopathy in a patient with hypothyroidism: Another element in ammonia metabolism","volume":"23","author":"D\u00edaz-Fontenla","year":"2017","journal-title":"World J Gastroenterol"},{"key":"R13-24-20240907","first-page":"275","article-title":"Renal impairment in patients with polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes syndrome: incidence, treatment and outcome","volume":"31","author":"Ye","year":"2016","journal-title":"Nephrol Dial Transplant"},{"key":"R14-24-20240907","doi-asserted-by":"crossref","first-page":"1319","DOI":"10.1046\/j.1440-1746.2001.02576.x","article-title":"Benign nodular hepatocellular lesions caused by abnormal hepatic circulation: Etiological analysis and introduction of a new concept","volume":"16","author":"Kondo","year":"2001","journal-title":"J Gastroenterol Hepatol"},{"key":"R15-24-20240907","doi-asserted-by":"crossref","first-page":"280","DOI":"10.1111\/cup.13415","article-title":"Tufted-angioma-like lesion associated with vascular endothelial growth factor and interleukin-6 in TAFRO syndrome: Is it a common histological feature of multicentric Castleman disease\/POEMS syndrome?","volume":"46","author":"Fujita","year":"2019","journal-title":"J Cutan Pathol"},{"key":"R16-24-20240907","doi-asserted-by":"crossref","first-page":"575","DOI":"10.1212\/WNL.0b013e318263c42b","article-title":"Markedly upregulated serum interleukin-12 as a novel biomarker in POEMS syndrome","volume":"79","author":"Kanai","year":"2012","journal-title":"Neurology"},{"key":"R17-24-20240907","doi-asserted-by":"crossref","first-page":"1318","DOI":"10.1038\/sj.leu.2400733","article-title":"POEMS syndrome: Report on six patients with unusual clinical signs, elevated levels of cytokines, macrophage involvement and chromosomal aberrations of bone marrow plasma cells","volume":"11","author":"Rose","year":"1997","journal-title":"Leukemia"},{"key":"R18-24-20240907","doi-asserted-by":"crossref","first-page":"e486","DOI":"10.1212\/NXI.0000000000000486","article-title":"Raised VEGF: High sensitivity and specificity in the diagnosis of POEMS syndrome","volume":"5","author":"Pihan","year":"2018","journal-title":"Neurol Neuroimmunol Neuroinflamm"}],"container-title":["Nigerian Journal of Clinical Practice"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.ovid.com\/10.4103\/njcp.njcp_360_22","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,8,26]],"date-time":"2026-08-26T19:38:27Z","timestamp":1787773107000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.ovid.com\/10.4103\/njcp.njcp_360_22"}},"subtitle":["a case report and literature review"],"short-title":[],"issued":{"date-parts":[[2022,11]]},"references-count":18,"journal-issue":{"issue":"11","published-print":{"date-parts":[[2022]]}},"URL":"https:\/\/doi.org\/10.4103\/njcp.njcp_360_22","relation":{},"ISSN":["1119-3077"],"issn-type":[{"value":"1119-3077","type":"print"}],"subject":[],"published":{"date-parts":[[2022,11]]}}}