{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,9]],"date-time":"2026-10-09T20:08:46Z","timestamp":1791576526539,"version":"4.3.4"},"reference-count":28,"publisher":"Wiley","issue":"9","license":[{"start":{"date-parts":[[2015,8,14]],"date-time":"2015-08-14T00:00:00Z","timestamp":1439510400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"name":"Deutsche Jos\u00e9 Carreras Leuk\u00e4mie Stiftung e.V.\u2032 (H11\/03), Germany"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["American J Hematol"],"published-print":{"date-parts":[[2015,9]]},"abstract":"<jats:p>Myeloproliferative neoplasms with eosinophilia are commonly characterized by a normal karyotype and remain poorly defined at the molecular level. We therefore investigated 426 samples from patients with hypereosinophilia of unknown significance initially referred for screening of the <jats:italic>FIP1L1\u2010PDGFRA<\/jats:italic> (<jats:italic>FP<\/jats:italic>) fusion gene also for <jats:italic>KIT<\/jats:italic> D816V and <jats:italic>JAK2<\/jats:italic> V617F mutations. Overall, 86 (20%) patients tested positive: <jats:italic>FP<\/jats:italic>+ in 55 (12%), <jats:italic>KIT<\/jats:italic> D816V+ in 14 (3%), and <jats:italic>JAK2<\/jats:italic> V617F+ in 17 (4%) patients, respectively. To gain better insight into clinical characteristics, we compared these cases with 31 additional and well\u2010characterized <jats:italic>KIT<\/jats:italic> D816V+ eosinophilia\u2010associated systemic mastocytosis (SM\u2010eo) patients enrolled within the \u201cGerman Registry on Disorders of Eosinophils and Mast cells.\u201d Significant differences included younger age, male predominance, and higher eosinophil counts for <jats:italic>FP<\/jats:italic>+\u2009cases while abdominal lymphadenopathy, ascites, and serum tryptase levels &gt;100 \u03bcg\/l were characteristic for those with <jats:italic>KIT<\/jats:italic> D816V. Leukocytes, hemoglobin, and splenomegaly did not differ significantly. A median of three additional mutations, most frequently <jats:italic>TET2<\/jats:italic> and <jats:italic>SRSF2<\/jats:italic>, were identified in 12\/13 <jats:italic>KIT<\/jats:italic> D816V+ SM\u2010eo patients with available material indicating a more complex molecular pathogenesis. Median survival was not reached for <jats:italic>FP<\/jats:italic>+ cases but was only 26 and 41 months for <jats:italic>KIT<\/jats:italic> D816V+ SM and <jats:italic>JAK2<\/jats:italic> V617F+ MPN\u2010eo, respectively. Eosinophilia of \u22652 \u00d7 10<jats:sup>9<\/jats:sup>\/l was identified as discriminator for inferior survival in <jats:italic>KIT<\/jats:italic> D816V+ and\/or <jats:italic>JAK2<\/jats:italic> V617F+ patients (median survival 20 months vs. not reached, <jats:italic>P<\/jats:italic>\u2009=\u20090.002). Thus, there is a clear prognostic and therapeutic rationale for detection of <jats:italic>KIT<\/jats:italic> D816V and <jats:italic>JAK2<\/jats:italic> V617F in the diagnostic work up of eosinophilia. Am. J. Hematol. 90:774\u2013777, 2015. \u00a9 2015 Wiley Periodicals, Inc.<\/jats:p>","DOI":"10.1002\/ajh.24075","type":"journal-article","created":{"date-parts":[[2015,5,28]],"date-time":"2015-05-28T05:20:40Z","timestamp":1432790440000},"page":"774-777","source":"Crossref","is-referenced-by-count":58,"title":["<scp><i>KIT<\/i><\/scp><scp><i>D<\/i><\/scp><i>816<\/i><scp><i>V<\/i><\/scp> and <scp><i>JAK<\/i><\/scp><i>2<\/i><scp><i>V<\/i><\/scp><i>617<\/i><scp><i>F<\/i><\/scp> mutations are seen recurrently in hypereosinophilia of unknown significance"],"prefix":"10.1002","volume":"90","author":[{"given":"Juliana","family":"Schwaab","sequence":"first","affiliation":[{"name":"Department of Hematology and Oncology University Hospital Mannheim Mannheim Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Roland","family":"Umbach","sequence":"additional","affiliation":[{"name":"Department of Hematology and Oncology University Hospital Mannheim Mannheim 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