{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,11]],"date-time":"2026-02-11T15:23:53Z","timestamp":1770823433268,"version":"3.50.1"},"reference-count":21,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2012,6,15]],"date-time":"2012-06-15T00:00:00Z","timestamp":1339718400000},"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":[[2012,7]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Objectives<\/jats:title><jats:p>The myeloproliferative neoplasms displaying a <jats:italic><jats:styled-content style=\"fixed-case\">PDGFRB<\/jats:styled-content><\/jats:italic> rearrangement are rare diseases derived from a haematopoietic stem cell. The goals of the study were to assess the incidence of these disorders and to define the clinical and biological characteristics as well as the response to the imatinib therapy.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>A total of 556 patients with myeloproliferative neoplasms were studied by means of molecular cytogenetics.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>The incidence of myeloproliferative neoplasms (<jats:styled-content style=\"fixed-case\">MPN<\/jats:styled-content>) with <jats:italic><jats:styled-content style=\"fixed-case\">PDGFRB<\/jats:styled-content><\/jats:italic> rearrangement was low (10 cases, 1.8% of all <jats:styled-content style=\"fixed-case\">MPN<\/jats:styled-content>). Most of the patients showed moderate anaemia (median Hb was 10.0 gr\/<jats:styled-content style=\"fixed-case\">dL<\/jats:styled-content>; range from 7.5 to 13\u00a0g\/<jats:styled-content style=\"fixed-case\">dL<\/jats:styled-content>), leukocytosis (median white blood cells was 21.7\u00a0\u00d7\u00a010<jats:sup>9<\/jats:sup>\/<jats:styled-content style=\"fixed-case\">L<\/jats:styled-content> with a range from 4 to 43\u00a0\u00d7\u00a010<jats:sup>9<\/jats:sup>\/<jats:styled-content style=\"fixed-case\">L<\/jats:styled-content>) and eosinophilia (median circulating eosinophils was 2.4\u00a0\u00d7\u00a010<jats:sup>9<\/jats:sup>\/<jats:styled-content style=\"fixed-case\">L<\/jats:styled-content> with a range of 1.1\u20135.7\u00a0\u00d7\u00a010<jats:sup>9<\/jats:sup>\/<jats:styled-content style=\"fixed-case\">L<\/jats:styled-content>) with a median of bone marrow infiltration cells displaying <jats:italic><jats:styled-content style=\"fixed-case\">PDGFRB<\/jats:styled-content><\/jats:italic> rearrangement of 55% (range, 37\u201385%). In three cases, a t(5;12) was observed while two patients showed rearrangements of 17q21 region. In two cases, a del(5)(q31) was observed. Most of the patients responded to standard dosage of imatinib, and the response was maintained in the time in those patients with a follow\u2010up higher than 9\u00a0years.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>The incidence of patients with <jats:italic><jats:styled-content style=\"fixed-case\">PDGFRB<\/jats:styled-content><\/jats:italic> rearrangement is low. These patients showed leukocytosis with eosinophilia and anaemia. The efficacy of imatinib therapy in patients showing <jats:italic><jats:styled-content style=\"fixed-case\">PDGFRB<\/jats:styled-content><\/jats:italic> rearrangement is high. For this reason, in all patients with <jats:styled-content style=\"fixed-case\">MPN<\/jats:styled-content> without any other molecular aberration, <jats:italic><jats:styled-content style=\"fixed-case\">PDGFRB<\/jats:styled-content><\/jats:italic> rearrangement should be ascertained.<\/jats:p><\/jats:sec>","DOI":"10.1111\/j.1600-0609.2012.01799.x","type":"journal-article","created":{"date-parts":[[2012,5,16]],"date-time":"2012-05-16T08:27:25Z","timestamp":1337156845000},"page":"37-41","source":"Crossref","is-referenced-by-count":37,"title":["Incidence and clinical characteristics of myeloproliferative neoplasms displaying a <i><scp>PDGFRB<\/scp><\/i> rearrangement"],"prefix":"10.1111","volume":"89","author":[{"given":"Maryam","family":"Arefi","sequence":"first","affiliation":[{"name":"Servicio de Hematolog\u00eda Hospital Cl\u00ednico Universitario de Valladolid Valladolid Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Juan L.","family":"Garc\u00eda","sequence":"additional","affiliation":[{"name":"Instituto de Ciencias de la Salud de Castilla y Le\u00f3n (IESCYL) Salamanca Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mar\u00eda J.","family":"Pe\u00f1arrubia","sequence":"additional","affiliation":[{"name":"Servicio de Hematolog\u00eda Hospital Cl\u00ednico Universitario de Valladolid Valladolid Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jos\u00e9 A.","family":"Queiz\u00e1n","sequence":"additional","affiliation":[{"name":"Servicio de Hematolog\u00eda Hospital General de Segovia Salamanca Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Lourdes","family":"Hermos\u00edn","sequence":"additional","affiliation":[{"name":"Servicio de Hematolog\u00eda Hospital del SAS Jerez 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