Sökning: onr:"swepub:oai:gup.ub.gu.se/310492" > Hematopoietic cell ...
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000 | 05887naa a2201021 4500 | |
001 | oai:gup.ub.gu.se/310492 | |
003 | SwePub | |
008 | 240528s2022 | |||||||||||000 ||eng| | |
009 | oai:prod.swepub.kib.ki.se:150362734 | |
024 | 7 | a https://gup.ub.gu.se/publication/3104922 URI |
024 | 7 | a https://doi.org/10.1016/j.jaci.2021.10.0172 DOI |
024 | 7 | a http://kipublications.ki.se/Default.aspx?queryparsed=id:1503627342 URI |
040 | a (SwePub)gud (SwePub)ki | |
041 | a eng | |
042 | 9 SwePub | |
072 | 7 | a ref2 swepub-contenttype |
072 | 7 | a art2 swepub-publicationtype |
100 | 1 | a Lankester, Arjan C4 aut |
245 | 1 0 | a Hematopoietic cell transplantation in severe combined immunodeficiency: The SCETIDE 2006-2014 European cohort. |
264 | 1 | b Elsevier BV,c 2022 |
520 | a Hematopoietic stem cell transplantation (HSCT) represents a curative treatment for patients with severe combined immunodeficiency (SCID), a group of monogenic immune disorders with an otherwise fatal outcome.We performed a comprehensive multicenter analysis of genotype-specific HSCT outcome, including detailed analysis of immune reconstitution (IR) and the predictive value for clinical outcome.HSCT outcome was studied in 338 patients with genetically confirmed SCID who underwent transplantation in 2006-2014 and who were registered in the SCETIDE registry. In a representative subgroup of 152 patients, data on IR and long-term clinical outcome were analyzed.Two-year OS was similar with matched family and unrelated donors and better than mismatched donor HSCT (P<.001). The 2-year event-free survival (EFS) was similar in matched and mismatched unrelated donor and less favorable in mismatched related donor (MMRD) HSCT (P< .001). Genetic subgroups did not differ in 2-year OS (P= .1) and EFS (P=.073). In multivariate analysis, pretransplantation infections and use of MMRDs were associated with less favorable OS and EFS. With a median follow-up of 6.2 years (range, 2.0-11.8 years), 73 of 152 patients in the IR cohort were alive and well without Ig dependency. IL-2 receptor gamma chain/Janus kinase 3/IL-7 receptor-deficient SCID, myeloablative conditioning, matched donor HSCT, and naive CD4 T lymphocytes >0.5×10e3/μL at+1 year were identified as independent predictors of favorable clinical and immunologic outcome.Recent advances in HSCT in SCID patients have resulted in improved OS and EFS in all genotypes and donor types. To achieve a favorable long-term outcome, treatment strategies should aim for optimal naive CD4 T lymphocyte regeneration. | |
650 | 7 | a MEDICIN OCH HÄLSOVETENSKAPx Klinisk medicinx Pediatrik0 (SwePub)302212 hsv//swe |
650 | 7 | a MEDICAL AND HEALTH SCIENCESx Clinical Medicinex Pediatrics0 (SwePub)302212 hsv//eng |
650 | 7 | a MEDICIN OCH HÄLSOVETENSKAPx Medicinska och farmaceutiska grundvetenskaperx Immunologi inom det medicinska området0 (SwePub)301102 hsv//swe |
650 | 7 | a MEDICAL AND HEALTH SCIENCESx Basic Medicinex Immunology in the medical area0 (SwePub)301102 hsv//eng |
700 | 1 | a Neven, Benedicte4 aut |
700 | 1 | a Mahlaoui, Nizar4 aut |
700 | 1 | a von Asmuth, Erik G J4 aut |
700 | 1 | a Courteille, Virginie4 aut |
700 | 1 | a Alligon, Mikael4 aut |
700 | 1 | a Albert, Michael H4 aut |
700 | 1 | a Serra, Isabelle Badell4 aut |
700 | 1 | a Bader, Peter4 aut |
700 | 1 | a Balashov, Dmitry4 aut |
700 | 1 | a Beier, Rita4 aut |
700 | 1 | a Bertrand, Yves4 aut |
700 | 1 | a Blanche, Stephane4 aut |
700 | 1 | a Bordon, Victoria4 aut |
700 | 1 | a Bredius, Robbert G4 aut |
700 | 1 | a Cant, Andrew4 aut |
700 | 1 | a Cavazzana, Marina4 aut |
700 | 1 | a Diaz-de-Heredia, Cristina4 aut |
700 | 1 | a Dogu, Figen4 aut |
700 | 1 | a Ehlert, Karoline4 aut |
700 | 1 | a Entz-Werle, Natacha4 aut |
700 | 1 | a Fasth, Anders,d 1945u Gothenburg University,Göteborgs universitet,Institutionen för kliniska vetenskaper, Avdelningen för pediatrik,Institute of Clinical Sciences, Department of Pediatrics4 aut0 (Swepub:gu)xfasan |
700 | 1 | a Ferrua, Francesca4 aut |
700 | 1 | a Ferster, Alina4 aut |
700 | 1 | a Formankova, Renata4 aut |
700 | 1 | a Friedrich, Wilhelm4 aut |
700 | 1 | a Gonzalez-Vicent, Marta4 aut |
700 | 1 | a Gozdzik, Jolanta4 aut |
700 | 1 | a Güngör, Tayfun4 aut |
700 | 1 | a Hoenig, Manfred4 aut |
700 | 1 | a Ikinciogullari, Aydan4 aut |
700 | 1 | a Kalwak, Krzysztof4 aut |
700 | 1 | a Kansoy, Savas4 aut |
700 | 1 | a Kupesiz, Alphan4 aut |
700 | 1 | a Lanfranchi, Arnalda4 aut |
700 | 1 | a Lindemans, Caroline A4 aut |
700 | 1 | a Meisel, Roland4 aut |
700 | 1 | a Michel, Gerard4 aut |
700 | 1 | a Miranda, Nuno A A4 aut |
700 | 1 | a Moraleda, Jose4 aut |
700 | 1 | a Moshous, Despina4 aut |
700 | 1 | a Pichler, Herbert4 aut |
700 | 1 | a Rao, Kanchan4 aut |
700 | 1 | a Sedlacek, Petr4 aut |
700 | 1 | a Slatter, Mary4 aut |
700 | 1 | a Soncini, Elena4 aut |
700 | 1 | a Speckmann, Carsten4 aut |
700 | 1 | a Sundin, Mikaelu Karolinska Institutet4 aut |
700 | 1 | a Toren, Amos4 aut |
700 | 1 | a Vettenranta, Kim4 aut |
700 | 1 | a Worth, Austen4 aut |
700 | 1 | a Yeşilipek, Mehmet A4 aut |
700 | 1 | a Zecca, Marco4 aut |
700 | 1 | a Porta, Fulvio4 aut |
700 | 1 | a Schulz, Ansgar4 aut |
700 | 1 | a Veys, Paul4 aut |
700 | 1 | a Fischer, Alain4 aut |
700 | 1 | a Gennery, Andrew R4 aut |
710 | 2 | a Göteborgs universitetb Institutionen för kliniska vetenskaper, Avdelningen för pediatrik4 org |
773 | 0 | t The Journal of allergy and clinical immunologyd : Elsevier BVg 149:5q 149:5x 1097-6825x 0091-6749 |
856 | 4 | u http://www.jacionline.org/article/S0091674921016298/pdf |
856 | 4 8 | u https://gup.ub.gu.se/publication/310492 |
856 | 4 8 | u https://doi.org/10.1016/j.jaci.2021.10.017 |
856 | 4 8 | u http://kipublications.ki.se/Default.aspx?queryparsed=id:150362734 |
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