{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,11]],"date-time":"2025-10-11T13:18:28Z","timestamp":1760188708982},"reference-count":20,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2006,4,26]],"date-time":"2006-04-26T00:00:00Z","timestamp":1146009600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["European J of Haematology"],"published-print":{"date-parts":[[2006,7]]},"abstract":"<jats:p><jats:bold>Abstract:\u2002<\/jats:bold>  <jats:italic>Purpose:<\/jats:italic>\u2002It is widely accepted that an increased serum endogenous erythropoietin (Epo) level in a patient presenting with an elevated red cell mass makes a diagnosis of clonal polycythemia vera (PV) extremely unlikely. However, until the recent description of the constitutively activating V617F point mutation of the Janus 2 tyrosine kinase (JAK2) \u2013 a high\u2010frequency molecular marker that is extremely specific for clonal chronic myeloproliferative disorders \u2013 distinction of PV from secondary erythrocytosis or other conditions has often been difficult. The purpose of this study was to use JAK2 V617F analysis to re\u2010evaluate the validity of elevated Epo levels as a PV\u2010exclusion criterion in patients with hepatic vein thrombosis [Budd\u2010Chiari syndrome (BCS)]. <jats:italic>Patients and methods:<\/jats:italic>\u2002We reviewed clinical data from 30 patients at our institution who presented with erythrocytosis and BCS. We isolated myeloid cells from fresh or archival bone marrow samples from four patients with BCS and an elevated serum Epo level, and analyzed them for the presence of the JAK2 V617F mutation. <jats:italic>Results:<\/jats:italic>\u2002All four samples were positive for JAK2 V617F, confirming the presence of a clonal hematopoietic disorder consistent with PV. <jats:italic>Conclusion:<\/jats:italic>\u2002In the presence of BCS, elevated serum Epo levels do not exclude the diagnosis of PV.<\/jats:p>","DOI":"10.1111\/j.1600-0609.2006.00667.x","type":"journal-article","created":{"date-parts":[[2006,4,26]],"date-time":"2006-04-26T15:32:50Z","timestamp":1146065570000},"page":"57-60","source":"Crossref","is-referenced-by-count":13,"title":["Elevated serum erythropoietin levels in patients with Budd\u2010Chiari syndrome secondary to polycythemia vera: clinical implications for the role of JAK2 mutation analysis"],"prefix":"10.1111","volume":"77","author":[{"given":"Paul J.","family":"Thurmes","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"David P.","family":"Steensma","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2006,4,26]]},"reference":[{"key":"e_1_2_7_2_2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM197610212951702"},{"key":"e_1_2_7_3_2","doi-asserted-by":"publisher","DOI":"10.4065\/78.2.174"},{"key":"e_1_2_7_4_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0037-1963(01)90136-2"},{"key":"e_1_2_7_5_2","volume-title":"Pathology and Genetics of Tumours of Haematopoietic and Lymphoid Tissues","author":"Pierre RVJ","year":"2001"},{"key":"e_1_2_7_6_2","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V99.4.1144"},{"key":"e_1_2_7_7_2","first-page":"1194","article-title":"Diagnostic value of serum erythropoietin level in patients with absolute erythrocytosis","volume":"89","author":"Mossuz P","year":"2004","journal-title":"Haematologica"},{"key":"e_1_2_7_8_2","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2141.2002.03386.x"},{"key":"e_1_2_7_9_2","doi-asserted-by":"publisher","DOI":"10.4065\/80.7.947"},{"key":"e_1_2_7_10_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(05)71142-9"},{"key":"e_1_2_7_11_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.ccr.2005.03.023"},{"key":"e_1_2_7_12_2","doi-asserted-by":"publisher","DOI":"10.1038\/nature03546"},{"key":"e_1_2_7_13_2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa051113"},{"key":"e_1_2_7_14_2","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.C500138200"},{"key":"e_1_2_7_15_2","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2005-03-1287"},{"key":"e_1_2_7_16_2","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2005-03-1320"},{"key":"e_1_2_7_17_2","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2005-03-1183"},{"key":"e_1_2_7_18_2","doi-asserted-by":"publisher","DOI":"10.1002\/hep.1840050525"},{"key":"e_1_2_7_19_2","doi-asserted-by":"publisher","DOI":"10.1001\/jama.205.10.696"},{"key":"e_1_2_7_20_2","doi-asserted-by":"publisher","DOI":"10.1177\/153537020322800101"},{"key":"e_1_2_7_21_2","doi-asserted-by":"publisher","DOI":"10.1097\/00041552-199601000-00007"}],"container-title":["European Journal of Haematology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fj.1600-0609.2006.00667.x","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/j.1600-0609.2006.00667.x","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,10,11]],"date-time":"2023-10-11T01:44:00Z","timestamp":1696988640000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/j.1600-0609.2006.00667.x"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2006,4,26]]},"references-count":20,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2006,7]]}},"alternative-id":["10.1111\/j.1600-0609.2006.00667.x"],"URL":"https:\/\/doi.org\/10.1111\/j.1600-0609.2006.00667.x","archive":["Portico"],"relation":{},"ISSN":["0902-4441","1600-0609"],"issn-type":[{"value":"0902-4441","type":"print"},{"value":"1600-0609","type":"electronic"}],"subject":[],"published":{"date-parts":[[2006,4,26]]}}}