{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,28]],"date-time":"2025-10-28T14:54:54Z","timestamp":1761663294859,"version":"3.40.4"},"reference-count":34,"publisher":"BMJ","content-domain":{"domain":["bmj.com"],"crossmark-restriction":true},"short-container-title":["BMJ Case Reports"],"accepted":{"date-parts":[[2014,11,12]]},"abstract":"<jats:p>Polycythaemia vera (PV) is a clonal disorder of bone marrow stem cells characterised by erythrocytosis. Diagnosis of PV requires exclusion of secondary causes of polycythaemia. It has been held that an elevated erythropoietin (Epo) level strongly indicates secondary erythrocytosis and excludes PV diagnosis, to the extent that the reduced serum Epo level is currently listed as a minor criterion in the WHO classification scheme for PV. However, patients with PV who co-present with Budd-Chiari syndrome have been documented with elevated serum Epo levels. For these patients, identification of the Janus kinase 2 (JAK2) V617F point mutation along with the transient nature of the Epo elevation provides certainty of PV diagnosis, as illustrated by the proband. In this case report, the patient's positive response to cytoreductive therapy (hydroxyurea 500\u2005mg daily) and phlebotomy (750\u2005mL over three phlebotomies) further supports validity of PV diagnosis with elevated Epo. The patient remains on rivaroxaban (Xarelto) for treatment of her portal vein thrombosis.<\/jats:p>","DOI":"10.1136\/bcr-2014-205663","type":"journal-article","created":{"date-parts":[[2014,12,1]],"date-time":"2014-12-01T17:22:00Z","timestamp":1417454520000},"page":"bcr2014205663","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":11,"title":["Elevated serum erythropoietin in a patient with polycythaemia vera presenting with Budd-Chiari syndrome"],"prefix":"10.1136","volume":"2014","author":[{"given":"Catherine","family":"Jones","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yair","family":"Levy","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Alex W","family":"Tong","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"239","published-online":{"date-parts":[[2014,12,1]]},"reference":[{"key":"2025042707041550000_2014.dec01_1.bcr2014205663.1","doi-asserted-by":"crossref","first-page":"285","DOI":"10.1002\/ajh.23135","article-title":"Polycythemia vera and essential thrombocythemia: 2012 update on diagnosis, risk stratification, and management","volume":"87","author":"Tefferi","year":"2012","journal-title":"Am J Hematol"},{"key":"2025042707041550000_2014.dec01_1.bcr2014205663.2","unstructured":"Swerdlow SH , Campo E , Harris NL , et al . eds. WHO classification of tumours of haematopoietic and lymphoid tissues. 4th edn. Lyon, France: IARC, 2008."},{"key":"2025042707041550000_2014.dec01_1.bcr2014205663.3","doi-asserted-by":"publisher","DOI":"10.1111\/j.1600-0609.2006.00667.x"},{"key":"2025042707041550000_2014.dec01_1.bcr2014205663.4","doi-asserted-by":"crossref","first-page":"794240","DOI":"10.1155\/2011\/794240","article-title":"The CYTO-PV: a large-scale trial testing the intensity of CYTOreductive therapy to prevent cardiovascular events in patients with polycythemia vera","volume":"2011","author":"Marchioli","year":"2011","journal-title":"Thrombosis"},{"key":"2025042707041550000_2014.dec01_1.bcr2014205663.5","doi-asserted-by":"publisher","DOI":"10.1111\/j.1478-3231.2008.01838.x"},{"key":"2025042707041550000_2014.dec01_1.bcr2014205663.6","doi-asserted-by":"crossref","first-page":"5","DOI":"10.52964\/AMJA.0439","article-title":"Budd-Chiari syndrome\u2014a review of the diagnosis and management","volume":"10","author":"Fox","year":"2011","journal-title":"Acute Med"},{"key":"2025042707041550000_2014.dec01_1.bcr2014205663.7","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2141.2002.03386.x"},{"key":"2025042707041550000_2014.dec01_1.bcr2014205663.8","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2014-05-578435"},{"key":"2025042707041550000_2014.dec01_1.bcr2014205663.9","first-page":"217","article-title":"Low serum erythropoietin--a strong diagnostic criterion of primary polycythaemia even at normal haemoglobin levels","volume":"17","author":"Messinezy","year":"1995","journal-title":"Clin Lab Haematol"},{"key":"2025042707041550000_2014.dec01_1.bcr2014205663.10","first-page":"1194","article-title":"Diagnostic value of serum erythropoietin level in patients with absolute erythrocytosis","volume":"89","author":"Mossuz","year":"2004","journal-title":"Haematologica"},{"key":"2025042707041550000_2014.dec01_1.bcr2014205663.11","doi-asserted-by":"publisher","DOI":"10.1007\/s00277-013-1988-6"},{"key":"2025042707041550000_2014.dec01_1.bcr2014205663.12","doi-asserted-by":"crossref","first-page":"485","DOI":"10.18632\/oncotarget.281","article-title":"Myeloproliferative neoplasms: from JAK2 mutations discovery to JAK2 inhibitor therapies","volume":"2","author":"Passamonti","year":"2011","journal-title":"Oncotarget"},{"key":"2025042707041550000_2014.dec01_1.bcr2014205663.13","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.23417"},{"key":"2025042707041550000_2014.dec01_1.bcr2014205663.14","doi-asserted-by":"publisher","DOI":"10.1002\/hep.1840050525"},{"key":"2025042707041550000_2014.dec01_1.bcr2014205663.15","doi-asserted-by":"publisher","DOI":"10.1002\/humu.22448"},{"key":"2025042707041550000_2014.dec01_1.bcr2014205663.16","doi-asserted-by":"publisher","DOI":"10.1080\/03630260600868147"},{"key":"2025042707041550000_2014.dec01_1.bcr2014205663.17","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2796.2008.02014.x"},{"key":"2025042707041550000_2014.dec01_1.bcr2014205663.18","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2008-12-196014"},{"key":"2025042707041550000_2014.dec01_1.bcr2014205663.19","doi-asserted-by":"publisher","DOI":"10.7326\/0003-4819-123-9-199511010-00003"},{"key":"2025042707041550000_2014.dec01_1.bcr2014205663.20","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2011-09-376517"},{"key":"2025042707041550000_2014.dec01_1.bcr2014205663.21","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2007-11-125328"},{"key":"2025042707041550000_2014.dec01_1.bcr2014205663.22","doi-asserted-by":"publisher","DOI":"10.1002\/hep.21435"},{"key":"2025042707041550000_2014.dec01_1.bcr2014205663.23","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(05)71142-9"},{"key":"2025042707041550000_2014.dec01_1.bcr2014205663.24","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2007-04-083501"},{"key":"2025042707041550000_2014.dec01_1.bcr2014205663.25","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2141.2005.05517.x"},{"key":"2025042707041550000_2014.dec01_1.bcr2014205663.26","doi-asserted-by":"publisher","DOI":"10.3324\/haematol.2012.067348"},{"key":"2025042707041550000_2014.dec01_1.bcr2014205663.27","doi-asserted-by":"publisher","DOI":"10.1097\/00041552-199601000-00007"},{"key":"2025042707041550000_2014.dec01_1.bcr2014205663.28","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1177\/153537020322800101","article-title":"Erythropoietin: physiology and pharmacology update","volume":"228","author":"Fisher","year":"2003","journal-title":"Exp Biol Med (Maywood)"},{"key":"2025042707041550000_2014.dec01_1.bcr2014205663.29","doi-asserted-by":"publisher","DOI":"10.3748\/wjg.v13.i19.2693"},{"key":"2025042707041550000_2014.dec01_1.bcr2014205663.30","doi-asserted-by":"publisher","DOI":"10.1038\/labinvest.2010.85"},{"key":"2025042707041550000_2014.dec01_1.bcr2014205663.31","doi-asserted-by":"publisher","DOI":"10.1172\/JCI30117"},{"key":"2025042707041550000_2014.dec01_1.bcr2014205663.32","doi-asserted-by":"publisher","DOI":"10.1002\/hep.26998"},{"key":"2025042707041550000_2014.dec01_1.bcr2014205663.33","doi-asserted-by":"publisher","DOI":"10.1160\/TH13-05-0407"},{"key":"2025042707041550000_2014.dec01_1.bcr2014205663.34","doi-asserted-by":"publisher","DOI":"10.1111\/jth.12485"}],"container-title":["BMJ Case Reports"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/syndication.highwire.org\/content\/doi\/10.1136\/bcr-2014-205663","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,4,27]],"date-time":"2025-04-27T14:04:23Z","timestamp":1745762663000},"score":1,"resource":{"primary":{"URL":"https:\/\/casereports.bmj.com\/lookup\/doi\/10.1136\/bcr-2014-205663"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2014,12,1]]},"references-count":34,"alternative-id":["10.1136\/bcr-2014-205663"],"URL":"https:\/\/doi.org\/10.1136\/bcr-2014-205663","relation":{},"ISSN":["1757-790X"],"issn-type":[{"type":"electronic","value":"1757-790X"}],"subject":[],"published":{"date-parts":[[2014,12,1]]}}}