{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,15]],"date-time":"2026-07-15T19:23:14Z","timestamp":1784143394836,"version":"3.55.0"},"reference-count":18,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2019,9,3]],"date-time":"2019-09-03T00:00:00Z","timestamp":1567468800000},"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":["J Paediatrics Child Health"],"published-print":{"date-parts":[[2020,3]]},"abstract":"<jats:sec><jats:title>Aim<\/jats:title><jats:p>Inborn errors of immunity (IEI) comprise a heterogeneous group of disorders of the immune system, most of which are curable by haematopoietic stem cell transplantation (HSCT). We present a 25\u2010year audit of HSCT for IEI at a tertiary\u2010level academic hospital in Malaysia.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Review of medical records of all cases of IEI who underwent HSCT between January 1993 and December 2018 at our centre. Diagnoses, complications, HSCT protocols and outcome data were studied.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>There were 20 patients (19 boys) with a median age at diagnosis of 11\u2009months (range: 2 months to 12\u2009years). Eleven of 19 (58%) had malnutrition at presentation. Donor sources were variable: 13 (65%) matched sibling donor (MSD), 4 (20%) human leukocyte antigen\u2010haploidentical donor (HD) and 3 (15%) matched unrelated donor (MUD). Conditioning regimens were physician\u2010dependent and adapted to each patient's clinical status. Grades III\u2013IV acute graft\u2010versus\u2010host disease occurred in two of three cases who received MUD grafts, 50% in those who received HD, and 8% in the MSD group. Transplant\u2010related mortality at day +100 was 5%. With a median follow\u2010up of 7.5\u2009years, 18 (90%) patients are alive and free of infections.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Outcome of HSCT for IEI in our centre is comparable with international reports. HSCT results using HD and MUD grafts are also good despite challenges from acute graft\u2010versus\u2010host disease, providing a feasible alternative for patients without matched donors.<\/jats:p><\/jats:sec>","DOI":"10.1111\/jpc.14621","type":"journal-article","created":{"date-parts":[[2019,9,3]],"date-time":"2019-09-03T19:51:44Z","timestamp":1567540304000},"page":"379-383","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":5,"title":["Haematopoietic stem cell transplantation for inborn errors of immunity: 25\u2010year experience from University of Malaya Medical Centre, Malaysia"],"prefix":"10.1111","volume":"56","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-4607-8837","authenticated-orcid":false,"given":"Hany","family":"Ariffin","sequence":"first","affiliation":[{"name":"Department of Paediatrics University of Malaya  Kuala Lumpur Malaysia"},{"name":"Paediatric Haematology\u2010Oncology and Bone Marrow Transplantation Unit University of Malaya Medical Centre  Kuala Lumpur Malaysia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Syaza","family":"Ab Rahman","sequence":"additional","affiliation":[{"name":"Paediatric Haematology\u2010Oncology and Bone Marrow Transplantation Unit University of Malaya Medical Centre  Kuala Lumpur Malaysia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vida","family":"Jawin","sequence":"additional","affiliation":[{"name":"Department of Paediatrics University of Malaya  Kuala Lumpur Malaysia"},{"name":"Paediatric Haematology\u2010Oncology and Bone Marrow Transplantation Unit University of Malaya Medical Centre  Kuala Lumpur Malaysia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jen Chun","family":"Foo","sequence":"additional","affiliation":[{"name":"Department of Paediatrics University of Malaya  Kuala Lumpur Malaysia"},{"name":"Paediatric Haematology\u2010Oncology and Bone Marrow Transplantation Unit University of Malaya Medical Centre  Kuala Lumpur Malaysia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nur Faizana","family":"Amram","sequence":"additional","affiliation":[{"name":"Paediatric Haematology\u2010Oncology and Bone Marrow Transplantation Unit University of Malaya Medical Centre  Kuala Lumpur Malaysia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nur Mahirah","family":"Mahmood","sequence":"additional","affiliation":[{"name":"Department of Paediatrics University of Malaya  Kuala Lumpur Malaysia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tsiao Yi","family":"Yap","sequence":"additional","affiliation":[{"name":"Department of Paediatrics University of Malaya  Kuala Lumpur Malaysia"},{"name":"Paediatric Haematology\u2010Oncology and Bone Marrow Transplantation Unit University of Malaya Medical Centre  Kuala Lumpur Malaysia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Revathi","family":"Rajagopal","sequence":"additional","affiliation":[{"name":"Department of Paediatrics University of Malaya  Kuala Lumpur Malaysia"},{"name":"Paediatric Haematology\u2010Oncology and Bone Marrow Transplantation Unit University of Malaya Medical Centre  Kuala Lumpur Malaysia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Su Han","family":"Lum","sequence":"additional","affiliation":[{"name":"Paediatric Haematology\u2010Oncology and Bone Marrow Transplantation Unit University of Malaya Medical Centre  Kuala Lumpur Malaysia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lee Lee","family":"Chan","sequence":"additional","affiliation":[{"name":"Subang Jaya Medical Centre  Subang Jaya Malaysia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hai Peng","family":"Lin","sequence":"additional","affiliation":[{"name":"Subang Jaya Medical Centre  Subang Jaya Malaysia"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2019,9,3]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10875-017-0464-9"},{"key":"e_1_2_7_3_1","doi-asserted-by":"publisher","DOI":"10.1084\/jem.20050854"},{"key":"e_1_2_7_4_1","first-page":"51","article-title":"Inborn errors of immunity: The missing link in infectious diseases susceptibility","volume":"4","author":"Pham\u2010Huy A","year":"2019","journal-title":"JAMMI"},{"key":"e_1_2_7_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.hoc.2014.08.006"},{"key":"e_1_2_7_6_1","doi-asserted-by":"publisher","DOI":"10.1080\/1744666X.2018.1424627"},{"key":"e_1_2_7_7_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1401177"},{"key":"e_1_2_7_8_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V99.3.872"},{"key":"e_1_2_7_9_1","doi-asserted-by":"publisher","DOI":"10.1038\/bmt.2008.121"},{"key":"e_1_2_7_10_1","unstructured":"Jeffrey Modell Foundation.The 10 Warning Signs of Primary Immunodeficiency. 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