{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,25]],"date-time":"2026-09-25T13:08:50Z","timestamp":1790341730608,"version":"4.1.0"},"reference-count":36,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2024,5,26]],"date-time":"2024-05-26T00:00:00Z","timestamp":1716681600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100003478","name":"Ministry of Health, Labour and Welfare","doi-asserted-by":"publisher","award":["20FC1005"],"award-info":[{"award-number":["20FC1005"]}],"id":[{"id":"10.13039\/501100003478","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Br J Haematol"],"published-print":{"date-parts":[[2024,9]]},"abstract":"<jats:title>Summary<\/jats:title><jats:p>Idiopathic hypereosinophilic syndrome (iHES) is a condition wherein persistent hypereosinophilia associated with end\u2010organ damage occurs without any known causes. Due to the rarity of the disease, insufficient knowledge has been accumulated. We therefore conducted a retrospective, multicentre, nationwide survey on iHES in Japan. A total of 57 patients were identified. For 43 patients who received any treatment, all cases were first treated with corticosteroids. An eosinophil percentage of less than 30% in the bone marrow and the absence of oedema were identified as factors associated with steroid dependency. The 5\u2010year overall survival was 88.2%, and five patients died during follow\u2010up; factors associated with worse overall survival were age &gt;50, haemoglobin &lt;12\u2009g\/dL, activated partial thromboplastin time &gt;34\u2009s, the presence of dyspnoea, the presence of thrombotic tendency and the presence of renal failure. Given the rarity of fatalities in our cohort, time\u2010to\u2010next\u2010treatment (TTNT) was further analysed; the presence of renal failure, splenomegaly and lung abnormalities were associated with worse TTNT. Our nationwide study not only demonstrated clinical characteristics and the outcome of patients with iHES but also for the first time revealed clinical factors associated with steroid dependency and duration of first\u2010line corticosteroid efficacy.<\/jats:p>","DOI":"10.1111\/bjh.19527","type":"journal-article","created":{"date-parts":[[2024,5,27]],"date-time":"2024-05-27T00:18:10Z","timestamp":1716769090000},"page":"967-977","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":5,"title":["Prognostic factors of idiopathic hypereosinophilic syndrome: A nationwide survey in Japan"],"prefix":"10.1111","volume":"205","author":[{"given":"Akira","family":"Honda","sequence":"first","affiliation":[{"name":"Department of Hematology and Oncology, Graduate School of Medicine The University of Tokyo  Tokyo 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