{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,7,30]],"date-time":"2025-07-30T10:04:20Z","timestamp":1753869860656,"version":"3.41.2"},"reference-count":20,"publisher":"Wiley","issue":"9","license":[{"start":{"date-parts":[[2020,7,29]],"date-time":"2020-07-29T00:00:00Z","timestamp":1595980800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatric Blood &amp; Cancer"],"published-print":{"date-parts":[[2020,9]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Hemophagocytic lymphohistiocytosis (HLH), a rare hyperinflammatory immuneregulatory disorder, is a challenge in hematopoietic stem cell transplantation (HSCT) because of the high rate of mixed chimerism, relapse, and graft failure (GF) unless intensive myeloablative regimens are used. However, historically conventional myeloablative regimens (conv\u00a0MA) are associated with high toxicity and mortality.<\/jats:p><\/jats:sec><jats:sec><jats:title>Procedure<\/jats:title><jats:p>We retrospectively compared transplant outcomes between three preparative regimens of varying intensities: Conv MA (n\u00a0=\u00a015), reduced\u2010intensity conditioning (RIC, n\u00a0=\u00a012), and a treosulfan\u2010based reduced\u2010toxicity conditioning (RTC, n\u00a0=\u00a09).<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Patients in the RIC cohort had a higher incidence of mixed donor chimerism and five patients (42%) developed secondary GF (<jats:italic>P<\/jats:italic>\u00a0=\u00a0.002) compared to the other two regimens. There was a higher incidence of veno\u2010occlusive disease and intensive care unit (ICU) admissions in the Conv MA cohort. With the RTC regimen, there was a similar 2\u2010year overall survival (89, 73, and 83%; <jats:italic>P<\/jats:italic>\u00a0=\u00a0.87), but improved compound EFS (lack of relapse, GF, second transplant or additional donor cell infusions, or death; 89, 73, and 42%, <jats:italic>P<\/jats:italic>\u00a0=\u00a0.041) in RTC, Conv MA, and RIC regimen, respectively.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>The intensity of the preparative regimen has a significant impact on outcome of HSCT for HLH. The newly described treosulfan\u2010based RTC provides for a stable graft with a reasonable toxicity profile.<\/jats:p><\/jats:sec>","DOI":"10.1002\/pbc.28477","type":"journal-article","created":{"date-parts":[[2020,7,29]],"date-time":"2020-07-29T15:02:54Z","timestamp":1596034974000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":8,"title":["Effect of different conditioning regimens on survival and engraftment for children with hemophagocytic lymphohistiocytosis undergoing allogeneic hematopoeitic stem cell transplantation: A single institution experience"],"prefix":"10.1002","volume":"67","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-3705-4064","authenticated-orcid":false,"given":"Salah","family":"Ali","sequence":"first","affiliation":[{"name":"Division of Hematology\/Oncology\/BMT Sheffield Childrens NHS Foundation Trust  Sheffield UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Donna A.","family":"Wall","sequence":"additional","affiliation":[{"name":"Division of Hematology\/Oncology, Department of Paediatrics The Hospital for Sick Children  Toronto Ontario Canada"},{"name":"Department of Pediatrics University of Toronto  Toronto Ontario Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Muhammad","family":"Ali","sequence":"additional","affiliation":[{"name":"Division of Hematology\/Oncology, Department of Paediatrics The Hospital for Sick Children  Toronto Ontario Canada"},{"name":"Department of Pediatrics University of Toronto  Toronto Ontario Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kuang\u2010Yueh","family":"Chiang","sequence":"additional","affiliation":[{"name":"Division of Hematology\/Oncology, Department of Paediatrics The Hospital for Sick Children  Toronto Ontario Canada"},{"name":"Department of Pediatrics University of Toronto  Toronto Ontario Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ahmed","family":"Naqvi","sequence":"additional","affiliation":[{"name":"Division of Hematology\/Oncology, Department of Paediatrics The Hospital for Sick Children  Toronto Ontario Canada"},{"name":"Department of Pediatrics University of Toronto  Toronto Ontario Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sheila","family":"Weitzman","sequence":"additional","affiliation":[{"name":"Division of Hematology\/Oncology, Department of Paediatrics The Hospital for Sick Children  Toronto Ontario Canada"},{"name":"Department of Pediatrics University of Toronto  Toronto Ontario Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Adam","family":"Gassas","sequence":"additional","affiliation":[{"name":"Pediatric Bone Marrow Transplant University Hospitals Bristol  Bristol UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Paul","family":"Gibson","sequence":"additional","affiliation":[{"name":"Pediatric Hematology\/Oncology, Children's Hospital London Health Sciences Centre  London Ontario Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Rae","family":"Brager","sequence":"additional","affiliation":[{"name":"Division of Rheumatology, Department of Pediatrics McMaster University Medical Centre  Hamilton Ontario Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Conrad V.","family":"Fernandez","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, IWK Health Centre Dalhousie University  Halifax Nova Scotia Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Bruce","family":"Crooks","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, IWK Health Centre Dalhousie University  Halifax Nova Scotia 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Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2020,7,29]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.1182\/asheducation-2011.1.178"},{"key":"e_1_2_7_3_1","doi-asserted-by":"publisher","DOI":"10.1038\/bmt.2008.133"},{"key":"e_1_2_7_4_1","doi-asserted-by":"publisher","DOI":"10.1111\/ctr.12069"},{"key":"e_1_2_7_5_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2010-04-282392"},{"key":"e_1_2_7_6_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2018-01-828277"},{"key":"e_1_2_7_7_1","doi-asserted-by":"publisher","DOI":"10.1200\/JCO.2009.23.3429"},{"key":"e_1_2_7_8_1","unstructured":"Childhood Hematopoietic Cell Transplantation [Internet]. 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