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Most studies report outcomes after bone marrow or peripheral blood stem cell transplantation. We analysed the outcomes of 118 children with <jats:styled-content style=\"fixed-case\">FHLH<\/jats:styled-content> undergoing single\u2010unit umbilical cord blood transplantation performed from 1996 to 2014. Myeloablative conditioning regimen was given to 90% of the patients, and was mostly busulfan\u2010based (<jats:italic>n<\/jats:italic>\u00a0=\u00a081, 76%), including anti\u2010thymocyte globulin or alemtuzumab (<jats:italic>n<\/jats:italic>\u00a0=\u00a0102, 86%). The cumulative incidence of Day 60 neutrophil engraftment was 85%; and that of non\u2010relapse mortality and acute graft\u2010versus\u2010host disease (Gv<jats:styled-content style=\"fixed-case\">HD<\/jats:styled-content>) was 21% and 33% at 100\u00a0days, respectively. The 6\u2010year cumulative incidence of chronic Gv<jats:styled-content style=\"fixed-case\">HD<\/jats:styled-content> was 17% and the 6\u2010year probability of overall survival was 55%. In multivariate analysis, children receiving a graft with a total nucleated cell dose greater than 9\u00b79\u00a0\u00d7\u00a010<jats:sup>7<\/jats:sup>\/kg had a better overall survival (hazard ratio [<jats:styled-content style=\"fixed-case\">HR<\/jats:styled-content>]: 0\u00b749, 95% <jats:styled-content style=\"fixed-case\">CI<\/jats:styled-content>: 0\u00b727\u20130\u00b788, <jats:italic>P\u00a0=\u00a0<\/jats:italic>0<jats:italic>\u00b7<\/jats:italic>02). Degree of human leucocyte antigen (<jats:styled-content style=\"fixed-case\">HLA<\/jats:styled-content>) matching was associated with improved disease\u2010free survival (5\/6 vs. 6\/6 <jats:styled-content style=\"fixed-case\">HR<\/jats:styled-content>: 2\u00b711, 95% confidence interval [<jats:styled-content style=\"fixed-case\">CI<\/jats:styled-content>]: 1\u00b701\u20134\u00b74, <jats:italic>P\u00a0=\u00a0<\/jats:italic>0<jats:italic>\u00b7<\/jats:italic>05 and \u22644\/6 vs. 6\/6, <jats:styled-content style=\"fixed-case\">HR<\/jats:styled-content>: 2\u00b782, <jats:styled-content style=\"fixed-case\">CI<\/jats:styled-content>: 1\u00b727\u20136\u00b723, <jats:italic>P\u00a0=\u00a0<\/jats:italic>0<jats:italic>\u00b7<\/jats:italic>01). Umbilical cord blood transplantation with a high cell dose and good <jats:styled-content style=\"fixed-case\">HLA<\/jats:styled-content> match is a suitable alternative option to haematopoietic stem cell transplantation in children with <jats:styled-content style=\"fixed-case\">FHLH<\/jats:styled-content> who lack a <jats:styled-content style=\"fixed-case\">HLA<\/jats:styled-content>\u2010matched donor.<\/jats:p>","DOI":"10.1111\/bjh.15642","type":"journal-article","created":{"date-parts":[[2018,11,21]],"date-time":"2018-11-21T10:13:00Z","timestamp":1542795180000},"page":"397-404","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":11,"title":["Risk factors affecting outcome of unrelated cord blood transplantation for children with familial haemophagocytic lymphohistiocytosis"],"prefix":"10.1111","volume":"184","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-1890-692X","authenticated-orcid":false,"given":"Juliana Montibeller","family":"Furtado\u2010Silva","sequence":"first","affiliation":[{"name":"Bone Marrow Transplant Department Great Ormond Street Hospital NHS Trust London UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Annalisa","family":"Paviglianiti","sequence":"additional","affiliation":[{"name":"Eurocord, H\u00f4pital Saint Louis Paris France"},{"name":"Monacord, Centre Scientifique de Monaco Monaco Monaco"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7261-2765","authenticated-orcid":false,"given":"Annalisa","family":"Ruggeri","sequence":"additional","affiliation":[{"name":"Eurocord, H\u00f4pital Saint Louis Paris France"},{"name":"Monacord, Centre Scientifique de Monaco Monaco Monaco"},{"name":"Department of Paediatric Haematology and Oncology Instituto di Ricovero e Cura a Carattere Scientifico (IRCCS) \u201cBambino Ges\u00f9\u201d Children's Hospital Rome Italy"},{"name":"Department of Paediatric Sciences University of Pavia Pavia Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jaap Jan","family":"Boelens","sequence":"additional","affiliation":[{"name":"Paediatric Blood and Marrow Transplantation Programme Paediatric Blood and Marrow Transplantation Programme University Hospital Utrecht Utrecht The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Paul","family":"Veys","sequence":"additional","affiliation":[{"name":"Bone Marrow Transplant Department Great Ormond Street Hospital NHS Trust London UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ali Abdallah","family":"Ahmari","sequence":"additional","affiliation":[{"name":"Department of Paediatric Haematology and Oncology King Faisal Specialist Hospital and Research Centre Riyadh Saudi 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Timone, CHU Marseille France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Fernanda","family":"Volt","sequence":"additional","affiliation":[{"name":"Eurocord, H\u00f4pital Saint Louis Paris France"},{"name":"Monacord, Centre Scientifique de Monaco Monaco Monaco"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Chantal","family":"Kenzey","sequence":"additional","affiliation":[{"name":"Eurocord, H\u00f4pital Saint Louis Paris France"},{"name":"Monacord, Centre Scientifique de Monaco Monaco Monaco"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Petr","family":"Sedlacek","sequence":"additional","affiliation":[{"name":"Department of Paediatric Haematology and Oncology University Hospital Motol Prague Czech Republic"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kanchan","family":"Rao","sequence":"additional","affiliation":[{"name":"Bone Marrow Transplant Department Great Ormond Street Hospital NHS Trust London 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