{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,27]],"date-time":"2026-05-27T20:01:30Z","timestamp":1779912090024,"version":"3.53.1"},"reference-count":45,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2024,10,10]],"date-time":"2024-10-10T00:00:00Z","timestamp":1728518400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"},{"start":{"date-parts":[[2024,10,10]],"date-time":"2024-10-10T00:00:00Z","timestamp":1728518400000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Br J Haematol"],"published-print":{"date-parts":[[2024,12]]},"abstract":"<jats:title>Summary<\/jats:title>\n                  <jats:p>\n                    The role of imatinib in\n                    <jats:italic>PDGFRA\/B<\/jats:italic>\n                    \u2010negative hypereosinophilic syndromes (HES) is controversial because of the heterogeneity of HES and the scarcity of prospective studies. We conducted a phase II clinical trial to evaluate the efficacy of imatinib in\n                    <jats:italic>PDGFRA\/B<\/jats:italic>\n                    \u2010negative HES. Thirty\u2010two patients were treated with imatinib (100\u2013400\u2009mg daily), and the molecular basis of their response was identified using whole\u2010exome sequencing (WES) and whole\u2010transcriptome sequencing (WTS). The haematological response rate was 46.9%, with a complete haematological response (CHR) rate of 18.8%. The median time to response was 1.5\u2009months. Among the six patients who achieved CHR, five maintained it until the 24th cycle of imatinib and one lost response after 20\u2009months. The median progression\u2010free survival was 4.3\u2009months. WES and WTS were conducted for 11 patients. The number of non\u2010silent mutations did not differ between responders and non\u2010responders. Nine differentially expressed genes, including\n                    <jats:italic>SNORD15A<\/jats:italic>\n                    , were downregulated in responders.\n                    <jats:italic>STAT5B<\/jats:italic>\n                    ::\n                    <jats:italic>RARA<\/jats:italic>\n                    ,\n                    <jats:italic>PAK2<\/jats:italic>\n                    ::\n                    <jats:italic>PIGX<\/jats:italic>\n                    , and\n                    <jats:italic>FIP1L1<\/jats:italic>\n                    ::\n                    <jats:italic>CHIC2<\/jats:italic>\n                    fusions were identified in patients with sustained responses, and\n                    <jats:italic>RNF130<\/jats:italic>\n                    ::\n                    <jats:italic>BRAF<\/jats:italic>\n                    and\n                    <jats:italic>WNK1<\/jats:italic>\n                    ::\n                    <jats:italic>KDM5A<\/jats:italic>\n                    fusions were identified in non\u2010responders. Imatinib, along with an appropriate biomarker, could be a promising option for\n                    <jats:italic>PDGFRA\/B<\/jats:italic>\n                    \u2010negative HES.\n                  <\/jats:p>","DOI":"10.1111\/bjh.19828","type":"journal-article","created":{"date-parts":[[2024,10,10]],"date-time":"2024-10-10T21:58:19Z","timestamp":1728597499000},"page":"2305-2314","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["Phase II trial of imatinib mesylate in patients with\n                    <i>PDGFRA\/B<\/i>\n                    \u2010negative hypereosinophilic syndrome"],"prefix":"10.1111","volume":"205","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-0369-5763","authenticated-orcid":false,"given":"Dong Hyun","family":"Kim","sequence":"first","affiliation":[{"name":"Department of Internal Medicine Seoul National University Hospital  Seoul Korea"},{"name":"Department of Translational Medicine Seoul National University College of Medicine  Seoul Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Seokhyeon","family":"Kim","sequence":"additional","affiliation":[{"name":"Cancer Research Institute Seoul National University College of Medicine  Seoul Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Seonyang","family":"Park","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine Inje University Haeundae Paik Hospital  Busan Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1780-5553","authenticated-orcid":false,"given":"Ja Min","family":"Byun","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine Seoul National University Hospital  Seoul Korea"},{"name":"Biomedical Research Institute Seoul National University Hospital  Seoul Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7829-397X","authenticated-orcid":false,"given":"Junshik","family":"Hong","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine Seoul National University Hospital  Seoul Korea"},{"name":"Cancer Research Institute Seoul National University College of Medicine  Seoul Korea"},{"name":"Biomedical Research Institute Seoul National University Hospital  Seoul Korea"},{"name":"Cancer for Medical Innovation Seoul National University Hospital  Seoul Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1753-8846","authenticated-orcid":false,"given":"Dong\u2010Yeop","family":"Shin","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine Seoul National University Hospital  Seoul Korea"},{"name":"Cancer Research Institute Seoul National University College of Medicine  Seoul Korea"},{"name":"Biomedical 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Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jeong\u2010Ok","family":"Lee","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine Seoul National University Bundang Hospital  Seongnam Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ji Yun","family":"Lee","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine Seoul National University Bundang Hospital  Seongnam Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8529-0232","authenticated-orcid":false,"given":"Sang\u2010A","family":"Kim","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine Seoul National University Bundang Hospital  Seongnam Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ki Hwan","family":"Kim","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine Seoul National University Boramae Hospital  Seoul 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