{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,28]],"date-time":"2026-08-28T12:31:32Z","timestamp":1787920292894,"version":"build-2784847793"},"reference-count":11,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2021,2,9]],"date-time":"2021-02-09T00:00:00Z","timestamp":1612828800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/501100004826","name":"Natural Science Foundation of Beijing Municipality","doi-asserted-by":"publisher","award":["7181003"],"award-info":[{"award-number":["7181003"]}],"id":[{"id":"10.13039\/501100004826","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001809","name":"National Natural Science Foundation of China","doi-asserted-by":"publisher","award":["81871633"],"award-info":[{"award-number":["81871633"]}],"id":[{"id":"10.13039\/501100001809","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":[[2021,5]]},"abstract":"<jats:title>Summary<\/jats:title><jats:p>We performed a multicentre, non\u2010randomised trial (NCT03533790) to investigate the efficacy of ruxolitinib combined with the doxorubicin\u2010etoposide\u2010methylprednisolone (Ru\u2010DEP) regimen as a salvage therapy for refractory\/relapsed haemophagocytic lymphohistiocytosis (HLH). All patients failing to achieve a complete or partial response 2 weeks after initial HLH\u201094\/HLH\u201004 regimen or relapsed after remission were enrolled in the study between June 2018 and June 2019. The efficacy was evaluated 2\u00a0weeks after initiating Ru\u2010DEP salvage therapy. Fifty\u2010four eligible patients with refractory\/relapsed (R\/R) HLH were enrolled. One case could not be evaluated for efficacy. Excluding 12 patients who had previously received the DEP regimen, the overall response rate was 32 of 41 (78\u00b70%) patients, with eight of 41 (19\u00b75%) achieving complete response and 24 of 41 (58\u00b75%) attaining a partial response. Of the R\/R HLH patients who had previously received the DEP regimen, 7 of 12 (58\u00b73%) achieved a partial response. Ferritin and soluble CD25 concentrations were significantly lower (<jats:italic>P<\/jats:italic>\u00a0&lt;\u00a00\u00b705), while the platelet count increased significantly (<jats:italic>P<\/jats:italic>\u00a0=\u00a00\u00b7034), and triglycerides decreased significantly (<jats:italic>P<\/jats:italic>\u00a0=\u00a00\u00b7002) compared with those before treatment. The Ru\u2010DEP regimen may be a safe and effective salvage therapy, remaining effective in refractory\/relapsed HLH following DEP treatment, especially in macrophage activation syndrome. In addition, the regimen can be considered for patients with contraindications to glucocorticoid, especially those with gastrointestinal bleeding.<\/jats:p>","DOI":"10.1111\/bjh.17331","type":"journal-article","created":{"date-parts":[[2021,2,10]],"date-time":"2021-02-10T21:22:08Z","timestamp":1612992128000},"page":"761-768","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":56,"title":["Ruxolitinib\u2010combined doxorubicin\u2010etoposide\u2010methylprednisolone regimen as a salvage therapy for refractory\/relapsed haemophagocytic lymphohistiocytosis: a single\u2010arm, multicentre, phase 2 trial"],"prefix":"10.1111","volume":"193","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-5050-9225","authenticated-orcid":false,"given":"Jingshi","family":"Wang","sequence":"first","affiliation":[{"name":"Department of Hematology Beijing Friendship Hospital Capital Medical University Beijing China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rui","family":"Zhang","sequence":"additional","affiliation":[{"name":"Hematology Oncology Center National Center for Children\u2019s Health Beijing Children\u2019s Hospital Capital Medical University Beijing China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xiaoyan","family":"Wu","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Union Hospital Tongji Medical College Huazhong University Of Science And Technology Hubei China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fei","family":"Li","sequence":"additional","affiliation":[{"name":"Department of Hematology The First Affiliated Hospital Of Nanchang University Jiangxi China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Haixia","family":"Yang","sequence":"additional","affiliation":[{"name":"Department of Hematology Hunan Children's Hospital Hunan China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ligen","family":"Liu","sequence":"additional","affiliation":[{"name":"Department of Hematology Tong Ren Hospital Shanghai Jiao Tong University School of Medicine Shanghai China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Haixia","family":"Guo","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Nanfang Hospital Southern Medical University Guangdong China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xuejun","family":"Zhang","sequence":"additional","affiliation":[{"name":"Department of Hematology The Second Hospital of Hebei Medical University Hebei China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Huirong","family":"Mai","sequence":"additional","affiliation":[{"name":"Department of Hematology\u2010Oncology Shenzhen Children's Hospital Guangdong China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hui","family":"Li","sequence":"additional","affiliation":[{"name":"Department of Hematology\u2010Oncology Wuhan Women and Children medical care center Hubei China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zhao","family":"Wang","sequence":"additional","affiliation":[{"name":"Department of Hematology Beijing Friendship Hospital Capital Medical University Beijing China"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2021,2,9]]},"reference":[{"key":"e_1_2_9_2_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2002-01-0172"},{"key":"e_1_2_9_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.cytogfr.2014.10.001"},{"key":"e_1_2_9_4_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2016-02-700013"},{"key":"e_1_2_9_5_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2015-12-684399"},{"key":"e_1_2_9_6_1","doi-asserted-by":"publisher","DOI":"10.3324\/haematol.2019.222471"},{"key":"e_1_2_9_7_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2015-05-644914"},{"key":"e_1_2_9_8_1","doi-asserted-by":"publisher","DOI":"10.1002\/pbc.21039"},{"key":"e_1_2_9_9_1","doi-asserted-by":"publisher","DOI":"10.1002\/pbc.24188"},{"key":"e_1_2_9_10_1","doi-asserted-by":"publisher","DOI":"10.1038\/nm.3645"},{"key":"e_1_2_9_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaad.2011.12.018"},{"key":"e_1_2_9_12_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1110556"}],"container-title":["British Journal of Haematology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/bjh.17331","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1111\/bjh.17331","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/bjh.17331","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,8,29]],"date-time":"2023-08-29T08:37:20Z","timestamp":1693298240000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/bjh.17331"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,2,9]]},"references-count":11,"journal-issue":{"issue":"4","published-print":{"date-parts":[[2021,5]]}},"alternative-id":["10.1111\/bjh.17331"],"URL":"https:\/\/doi.org\/10.1111\/bjh.17331","archive":["Portico"],"relation":{},"ISSN":["0007-1048","1365-2141"],"issn-type":[{"value":"0007-1048","type":"print"},{"value":"1365-2141","type":"electronic"}],"subject":[],"published":{"date-parts":[[2021,2,9]]},"assertion":[{"value":"2020-10-04","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2020-12-23","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2021-02-09","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}