{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,1]],"date-time":"2026-05-01T12:30:36Z","timestamp":1777638636941,"version":"3.51.4"},"reference-count":44,"publisher":"Wiley","issue":"8","license":[{"start":{"date-parts":[[2015,3,31]],"date-time":"2015-03-31T00:00:00Z","timestamp":1427760000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Liver International"],"published-print":{"date-parts":[[2015,8]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>A considerable proportion of patients receiving liver transplants for Budd\u2010Chiari syndrome (<jats:styled-content style=\"fixed-case\">BCS<\/jats:styled-content>) suffer from myeloproliferative neoplasms (<jats:styled-content style=\"fixed-case\">MPN<\/jats:styled-content>). This study evaluated the long\u2010term prognosis of liver\u2010transplanted patients with<jats:styled-content style=\"fixed-case\">BCS<\/jats:styled-content>secondary to<jats:styled-content style=\"fixed-case\">MPN<\/jats:styled-content>and the effect of immunosuppression on<jats:styled-content style=\"fixed-case\">MPN<\/jats:styled-content>progression.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>A total of 78 patients with<jats:styled-content style=\"fixed-case\">BCS<\/jats:styled-content>were evaluated between 1982 and 2013. Of those, 40 patients suffered from polycythaemia vera (<jats:styled-content style=\"fixed-case\">PV<\/jats:styled-content>) and essential thrombocythaemia (<jats:styled-content style=\"fixed-case\">ET<\/jats:styled-content>). One patient had primary myelofibrosis (<jats:styled-content style=\"fixed-case\">PMF<\/jats:styled-content>). All patients received the standard immunosuppressive regimen. We retrospectively evaluated the long\u2010term survival, clinical course and laboratory parameters of patients with<jats:styled-content style=\"fixed-case\">MPN<\/jats:styled-content>.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Exactly 29\/41 patients (71%) with<jats:styled-content style=\"fixed-case\">MPN<\/jats:styled-content>survived \u2265\u00a03\u00a0years [mean age 36\u00a0\u00b1\u00a011\u00a0years; females<jats:italic>n<\/jats:italic>\u00a0=\u00a027 (93%)]. Mean follow\u2010up after orthotopic liver transplantation (<jats:styled-content style=\"fixed-case\">OLT<\/jats:styled-content>) was 12.4\u00a0\u00b1\u00a07.3\u00a0years (range 3\u201328\u00a0years). Five\u2010 and 10\u2010year survival rates were not significantly different in patients with and without<jats:styled-content style=\"fixed-case\">MPN<\/jats:styled-content>(<jats:italic>P<\/jats:italic>\u00a0=\u00a00.81 and<jats:italic>P<\/jats:italic>\u00a0=\u00a00.66 respectively) or in patients with<jats:styled-content style=\"fixed-case\">PV<\/jats:styled-content>and<jats:styled-content style=\"fixed-case\">ET<\/jats:styled-content>(<jats:italic>P<\/jats:italic>\u00a0=\u00a00.29 and<jats:italic>P<\/jats:italic>\u00a0=\u00a00.55 respectively). Thrombosis and bleeding developed in 7\/29 (24%) long\u2010term<jats:styled-content style=\"fixed-case\">MPN<\/jats:styled-content>survivors with no significant difference between<jats:styled-content style=\"fixed-case\">ET<\/jats:styled-content>and<jats:styled-content style=\"fixed-case\">PV<\/jats:styled-content>(<jats:italic>P<\/jats:italic>\u00a0=\u00a00.18). In the long\u2010term follow\u2010up, there was no evidence of progression to overt myelofibrosis or acute myeloid leukaemia (<jats:styled-content style=\"fixed-case\">AML<\/jats:styled-content>). In the uni\u2010 and multivariate Cox\u2010regression analyses,<jats:styled-content style=\"fixed-case\">MPN<\/jats:styled-content>did not influence survival after<jats:styled-content style=\"fixed-case\">OLT<\/jats:styled-content>.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Budd\u2010Chiari syndrome patients with and without underlying<jats:styled-content style=\"fixed-case\">MPN<\/jats:styled-content>had similar long\u2010term survival rates after<jats:styled-content style=\"fixed-case\">OLT<\/jats:styled-content>. There was no evidence of enhanced progression of<jats:styled-content style=\"fixed-case\">MPN<\/jats:styled-content>after<jats:styled-content style=\"fixed-case\">OLT<\/jats:styled-content>secondary to immunosuppressive therapy. However, major haemorrhage and recurrent thrombosis contributed to morbidity and mortality after<jats:styled-content style=\"fixed-case\">OLT<\/jats:styled-content>in those patients.<\/jats:p><\/jats:sec>","DOI":"10.1111\/liv.12816","type":"journal-article","created":{"date-parts":[[2015,3,3]],"date-time":"2015-03-03T14:26:53Z","timestamp":1425392813000},"page":"2042-2049","source":"Crossref","is-referenced-by-count":40,"title":["Long\u2010term outcome of liver transplant patients with Budd\u2010Chiari syndrome secondary to myeloproliferative neoplasms"],"prefix":"10.1111","volume":"35","author":[{"given":"Andrej","family":"Potthoff","sequence":"first","affiliation":[{"name":"Department of Gastroenterology, Hepatology and Endocrinology Hannover Medical School Hannover Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Dina","family":"Attia","sequence":"additional","affiliation":[{"name":"Department of Gastroenterology, Hepatology and Endocrinology Hannover Medical 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