{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,9]],"date-time":"2026-07-09T08:57:35Z","timestamp":1783587455041,"version":"3.55.0"},"reference-count":19,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2024,5,3]],"date-time":"2024-05-03T00:00:00Z","timestamp":1714694400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Med."],"abstract":"<jats:p>Primary myelofibrosis (PMF) is an infrequent etiology of noncirrhotic portal hypertension (PH). In clinical settings, non-cirrhotic PH is often misdiagnosed as cirrhotic PH. This case report details a patient who exhibited recurrent esophageal variceal hemorrhage and was initially misdiagnosed with cirrhosis. Initially poised for liver transplantation, the patient\u2019s liver biopsy revealed no significant cirrhosis but showed signs of extramedullary hematopoiesis (EMH). Following the accurate diagnosis of PMF, the patient underwent standard treatment, leading to an absence of recurrent gastrointestinal hemorrhage due to esophageal varices for nearly three years.<\/jats:p>","DOI":"10.3389\/fmed.2024.1375571","type":"journal-article","created":{"date-parts":[[2024,5,3]],"date-time":"2024-05-03T04:20:34Z","timestamp":1714710034000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":4,"title":["Case report: Primary myelofibrosis presenting with portal hypertension mimicking cirrhosis"],"prefix":"10.3389","volume":"11","author":[{"given":"Xiayu","family":"Gan","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shengjie","family":"Yu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Min","family":"Zhu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bo","family":"Ning","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Song","family":"He","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xiaoyue","family":"Xie","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Linshuang","family":"Tu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Huihong","family":"Yu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1965","published-online":{"date-parts":[[2024,5,3]]},"reference":[{"key":"ref1","doi-asserted-by":"publisher","first-page":"8520","DOI":"10.1200\/JCO.2004.00.9316","article-title":"Pathogenesis of myelofibrosis with myeloid metaplasia","volume":"23","author":"Tefferi","year":"2005","journal-title":"J Clin Oncol"},{"key":"ref2","doi-asserted-by":"publisher","first-page":"11","DOI":"10.1111\/jgh.13038","article-title":"Education and imaging. Hepatobiliary and pancreatic: a rare cause of portal hypertension","volume":"31","author":"Plaz Torres","year":"2016","journal-title":"J Gastroenterol Hepatol"},{"key":"ref3","doi-asserted-by":"publisher","first-page":"532","DOI":"10.1001\/archinte.1973.00320100060007","article-title":"Gastrointestinal and abdominal manifestations of agnogenic myeloid metaplasia","volume":"131","author":"Silverstein","year":"1973","journal-title":"Arch Intern Med"},{"key":"ref4","doi-asserted-by":"publisher","first-page":"2391","DOI":"10.1182\/blood-2016-03-643544","article-title":"The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia","volume":"127","author":"Arber","year":"2016","journal-title":"Blood"},{"key":"ref5","doi-asserted-by":"publisher","first-page":"312","DOI":"10.1111\/j.1751-2980.2011.00514.x","article-title":"Diagnosis and treatment of portal hypertension secondary to myeloproliferative disorders: a report of three cases","volume":"12","author":"Zhao","year":"2011","journal-title":"J Dig Dis"},{"key":"ref6","doi-asserted-by":"publisher","first-page":"81","DOI":"10.1111\/j.1600-0609.1978.tb02497.x","article-title":"Nature and incidence of liver involvement in agnogenic myeloid metaplasia","volume":"21","author":"Ligumski","year":"1978","journal-title":"Scand J Haematol"},{"key":"ref7","doi-asserted-by":"publisher","first-page":"1067","DOI":"10.1016\/0016-5085(87)90984-X","article-title":"Agnogenic myeloid metaplasia, portal hypertension, and sinusoidal abnormalities","volume":"92","author":"Roux","year":"1987","journal-title":"Gastroenterology"},{"key":"ref8","doi-asserted-by":"publisher","first-page":"246","DOI":"10.1002\/hep.1840170214","article-title":"Portal hypertension in lymphoproliferative and myeloproliferative disorders: hemodynamic and histological correlations","volume":"17","author":"Dubois","year":"1993","journal-title":"Hepatology"},{"key":"ref9","doi-asserted-by":"publisher","first-page":"915","DOI":"10.1023\/A:1010737409137","article-title":"Esophageal variceal ligation for esophageal variceal hemorrhage in a patient with portal and primary pulmonary hypertension complicating myelofibrosis","volume":"46","author":"Lee","year":"2001","journal-title":"Dig Dis Sci"},{"key":"ref10","doi-asserted-by":"publisher","first-page":"355","DOI":"10.1111\/j.1600-0609.1988.tb00191.x","article-title":"Liver involvement at diagnosis of primary myelofibrosis: a clinicopathological study of twenty-two cases","volume":"40","author":"Pereira","year":"1988","journal-title":"Eur J Haematol"},{"key":"ref11","doi-asserted-by":"publisher","first-page":"121","DOI":"10.1054\/blre.2000.0132","article-title":"Splenectomy in chronic myeloid leukemia and myelofibrosis with myeloid metaplasia","volume":"14","author":"Mesa","year":"2000","journal-title":"Blood Rev"},{"key":"ref12","doi-asserted-by":"publisher","first-page":"2355","DOI":"10.1111\/j.1572-0241.2005.50374.x","article-title":"Portal hypertension secondary to myelofibrosis: a study of three cases","volume":"100","author":"Alvarez-Larran","year":"2005","journal-title":"Am J Gastroenterol"},{"key":"ref13","doi-asserted-by":"publisher","first-page":"45","DOI":"10.1159\/000206164","article-title":"Myelofibrosis, splenomegaly, and portal hypertension","volume":"74","author":"Jacobs","year":"1985","journal-title":"Acta Haematol"},{"key":"ref14","doi-asserted-by":"publisher","first-page":"4246","DOI":"10.1182\/blood-2002-01-0282","article-title":"Transjugular intrahepatic portosystemic shunt for treatment of portal hypertension due to extramedullary hematopoiesis in idiopathic myelofibrosis","volume":"99","author":"Angermayr","year":"2002","journal-title":"Blood"},{"key":"ref15","doi-asserted-by":"publisher","first-page":"217854","DOI":"10.1136\/bcr-2016-217854","article-title":"Idiopathic myelofibrosis with disseminated hepatosplenic, mesenteric, renal, and pulmonary extramedullary haematopoeisis, portal hypertension, and tuberculosis: initial presentation and 2 years follow-up","volume":"2016","author":"Panda","year":"2016","journal-title":"BMJ Case Rep"},{"key":"ref16","first-page":"771","article-title":"Portal hypertension complicating myelofibrosis: reversal following splenectomy","volume":"117","author":"Lukie","year":"1977","journal-title":"Can Med Assoc J"},{"key":"ref17","doi-asserted-by":"publisher","first-page":"389","DOI":"10.1080\/00365520310007521","article-title":"A patient with myelofibrosis complicated by refractory ascites and portal hypertension: to TIPS or not to TIPS? A case report with discussion of the mechanism of ascites formation","volume":"39","author":"Wiest","year":"2004","journal-title":"Scand J Gastroenterol"},{"key":"ref18","doi-asserted-by":"publisher","first-page":"3770","DOI":"10.3748\/wjg.v18.i28.3770","article-title":"Multiple esophageal variceal ruptures with massive ascites due to myelofibrosis-induced portal hypertension","volume":"18","author":"Tokai","year":"2012","journal-title":"World J Gastroenterol"},{"key":"ref19","doi-asserted-by":"publisher","first-page":"e00333","DOI":"10.14309\/crj.0000000000000333","article-title":"Myelofibrosis and portal hypertension: the case for primary variceal screening","volume":"7","author":"Sherman","year":"2020","journal-title":"ACG Case Rep J"}],"container-title":["Frontiers in Medicine"],"original-title":[],"link":[{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fmed.2024.1375571\/full","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,5,3]],"date-time":"2024-05-03T04:20:37Z","timestamp":1714710037000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fmed.2024.1375571\/full"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,5,3]]},"references-count":19,"alternative-id":["10.3389\/fmed.2024.1375571"],"URL":"https:\/\/doi.org\/10.3389\/fmed.2024.1375571","relation":{},"ISSN":["2296-858X"],"issn-type":[{"value":"2296-858X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2024,5,3]]},"article-number":"1375571"}}