{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,12]],"date-time":"2026-10-12T16:28:21Z","timestamp":1791822501944,"version":"4.3.4"},"reference-count":17,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2024,5,13]],"date-time":"2024-05-13T00:00:00Z","timestamp":1715558400000},"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":["Scand J Immunol"],"published-print":{"date-parts":[[2024,8]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Autoimmune cytopenias are a heterogeneous group of disorders characterized by immune\u2010mediated destruction of haematopoietic cell lines. Effective and well\u2010tolerated treatment options for relapsed\u2010refractory immune cytopenias are limited. In this study, the aim was to evaluate the efficacy and safety of sirolimus in this disease group within the paediatric age group. The study enrolled patients in the paediatric age group who used sirolimus with a diagnosis of immune cytopenia between December 2010 and December 2020, followed at six centres in Turkey. Of the 17 patients, five (29.4%) were treated for autoimmune haemolytic anaemia (AIHA), six (35.2%) for immune thrombocytopenic purpura (ITP) and six (35.2%) for Evans syndrome (ES). The mean response time was 2.7\u2009months (range, 0\u20139\u2009months). Complete response (CR) and partial response (PR) were obtained in 13 of 17 patients (76.4%) and nonresponse (NR) in four patients (23.5%). Among the 13 patients who achieved CR, three of them were NR in the follow\u2010up and two of them had remission with low\u2010dose steroid and sirolimus. Thus, overall response rate (ORR) was achieved in 12 of 17 patients (70.5%). In conclusion, sirolimus may be an effective and safe option in paediatric patients with relapsed\u2010refractory immune cytopenia.<\/jats:p>","DOI":"10.1111\/sji.13376","type":"journal-article","created":{"date-parts":[[2024,5,14]],"date-time":"2024-05-14T03:44:55Z","timestamp":1715658295000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":6,"title":["Sirolimus is effective and safe in childhood relapsed\u2010refractory autoimmune cytopenias: A multicentre study"],"prefix":"10.1111","volume":"100","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-5768-0890","authenticated-orcid":false,"given":"Sultan Okur","family":"Acar","sequence":"first","affiliation":[{"name":"Deparment of Hematology and Oncology University of Health Sciences Dr. Beh\u00e7et Uz Children's Hospital  Izmir Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Neryal","family":"Tahta","sequence":"additional","affiliation":[{"name":"Deparment of Hematology and Oncology University of Health Sciences Dr. Beh\u00e7et Uz Children's Hospital  Izmir Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"I\u015f\u0131k Odaman","family":"Al","sequence":"additional","affiliation":[{"name":"Deparment of Hematology and Oncology University of Health Sciences Dr. Beh\u00e7et Uz Children's Hospital  Izmir Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Melek","family":"Erdem","sequence":"additional","affiliation":[{"name":"Deparment of Hematology and Oncology University of Health Sciences Dr. Beh\u00e7et Uz Children's Hospital  Izmir Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Salih","family":"G\u00f6zmen","sequence":"additional","affiliation":[{"name":"Deparment of Hematology and Oncology University of Health Sciences Dr. Beh\u00e7et Uz Children's Hospital  Izmir Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tuba Hilkay","family":"Karap\u0131nar","sequence":"additional","affiliation":[{"name":"Deparment of Hematology and Oncology University of Health Sciences Dr. Beh\u00e7et Uz Children's Hospital  Izmir Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Burcu","family":"K\u0131l\u0131n\u00e7","sequence":"additional","affiliation":[{"name":"Faculty of Medicine Hospital Istanbul University Cerrahpa\u015fa  Istanbul Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tiraje","family":"Celkan","sequence":"additional","affiliation":[{"name":"Faculty of Medicine Hospital Istanbul University Cerrahpa\u015fa  Istanbul Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Serap","family":"Kirkiz","sequence":"additional","affiliation":[{"name":"Faculty of Medicine Hospital Ankara Gazi University  Ankara Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"\u00dclker","family":"Ko\u00e7ak","sequence":"additional","affiliation":[{"name":"Faculty of Medicine Hospital Ankara Gazi University  Ankara Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hale","family":"\u00d6ren","sequence":"additional","affiliation":[{"name":"Faculty of Medicine Hospital Dokuz Eyl\u00fcl University  Izmir Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ay\u015fen T\u00fcredi","family":"Y\u0131ld\u0131r\u0131m","sequence":"additional","affiliation":[{"name":"Faculty of Medicine Hospital Manisa Celal Bayar University  Manisa Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9549-5039","authenticated-orcid":false,"given":"Esra","family":"Arslanta\u015f","sequence":"additional","affiliation":[{"name":"Ba\u015fak\u015fehir \u00c7am and Sakura City Hospital  Istanbul Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Aylin Canbolat","family":"Ayhan","sequence":"additional","affiliation":[{"name":"Medeniyet Univercity G\u00f6ztepe Training and Research Hospital  Istanbul Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ye\u015fim","family":"Oymak","sequence":"additional","affiliation":[{"name":"Deparment of Hematology and Oncology University of Health Sciences Dr. Beh\u00e7et Uz Children's Hospital  Izmir Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2024,5,13]]},"reference":[{"key":"e_1_2_13_2_1","doi-asserted-by":"publisher","DOI":"10.1111\/bjh.13533"},{"key":"e_1_2_13_3_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2014-06-583260"},{"key":"e_1_2_13_4_1","doi-asserted-by":"publisher","DOI":"10.1111\/bjh.13866"},{"key":"e_1_2_13_5_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.0902-4441.2006.t01-1-EJH2549.x"},{"key":"e_1_2_13_6_1","unstructured":"Food and Drug Administration.FDA approves rapamune to prevent organ rejection.http:\/\/www.fda.gov\/bbs\/topics\/ANSWERS\/ANS00974.html"},{"key":"e_1_2_13_7_1","doi-asserted-by":"publisher","DOI":"10.1542\/peds.2015-3257"},{"key":"e_1_2_13_8_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2015-07-657981"},{"key":"e_1_2_13_9_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.it.2018.04.006"},{"key":"e_1_2_13_10_1","doi-asserted-by":"publisher","DOI":"10.4049\/jimmunol.1301181"},{"key":"e_1_2_13_11_1","doi-asserted-by":"publisher","DOI":"10.1155\/2013\/548085"},{"key":"e_1_2_13_12_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2011-07-325217"},{"key":"e_1_2_13_13_1","doi-asserted-by":"publisher","DOI":"10.1097\/MPH.0000000000000818"},{"key":"e_1_2_13_14_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.exphem.2020.08.001"},{"key":"e_1_2_13_15_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2017-09-742353"},{"key":"e_1_2_13_16_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMcp1810479"},{"key":"e_1_2_13_17_1","doi-asserted-by":"publisher","DOI":"10.3389\/fmed.2020.00110"},{"key":"e_1_2_13_18_1","doi-asserted-by":"publisher","DOI":"10.1111\/bjh.13010"}],"container-title":["Scandinavian Journal of Immunology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/sji.13376","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,7,15]],"date-time":"2024-07-15T06:58:48Z","timestamp":1721026728000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/sji.13376"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,5,13]]},"references-count":17,"journal-issue":{"issue":"2","published-print":{"date-parts":[[2024,8]]}},"alternative-id":["10.1111\/sji.13376"],"URL":"https:\/\/doi.org\/10.1111\/sji.13376","archive":["Portico"],"relation":{},"ISSN":["0300-9475","1365-3083"],"issn-type":[{"value":"0300-9475","type":"print"},{"value":"1365-3083","type":"electronic"}],"subject":[],"published":{"date-parts":[[2024,5,13]]},"assertion":[{"value":"2023-09-18","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2024-03-24","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2024-05-13","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}],"article-number":"e13376"}}