Primary immunodeficiencies: HSCT experiences of a single center in Turkey

dc.authoridCeyhun Bozkurt / 0000-0001-6771-9894en_US
dc.authoridFunda Erol Çipe / 0000-0002-9718-7507
dc.authorscopusidCeyhun Bozkurt / 6602784315
dc.authorscopusidFunda Erol Çipe / 25824763200
dc.authorwosidCeyhun Bozkurt / AAL-5043-2021
dc.authorwosidFunda Erol Çipe / GDE-8701-2022
dc.contributor.authorErol Çipe, Funda
dc.contributor.authorAdaklı Aksoy, Başak
dc.contributor.authorAydoğdu, Selime
dc.contributor.authorDikme, Gürcan
dc.contributor.authorKıykım, Ayca
dc.contributor.authorAydoğmus, Çiğdem
dc.contributor.authorYücel, Esra
dc.contributor.authorBozkurt, Ceyhun
dc.contributor.authorFışgın, Tunç
dc.date.accessioned2021-06-11T10:44:58Z
dc.date.available2021-06-11T10:44:58Z
dc.date.issued2021en_US
dc.departmentİstinye Üniversitesi, Tıp Fakültesi, Dahili Tıp Bilimleri Bölümüen_US
dc.description.abstractBackground Primary immunodeficiency diseases (PID) are characterized by the occurrence of frequent infections and are caused by many genetic defects. Hematopoietic stem cell transplantation (HSCT) is the only curative treatment option for the majority of PID. As a Pediatric Hematology-Oncology-Immunology Transplantation Unit, we wanted to present our HSCT experience regarding treatment of primary immunodeficiency diseases. Methods 58 patients were included in the study between January 2014 and June 2019. We searched 9/10 or 10/10 matched-related donor (MRD) firstly, in the absence of fully matched-related donor. We screened matched unrelated donor (MUD) from donor banks. MRD was used in 24 (41.3%) patients, MUD in 20 (34.4%) patients, and haploidentical donors in 14 (24.1%) patients. Demographic data, HSCT characteristics, and outcome were evaluated. While 16 patients had severe combined immunodeficiency (SCID), the remaining was non-SCID. Results Of the 58 patients, 38 were male and 20 were female. Median age at transplantation was 12 months (range: 2.5-172 months). Combined immunodeficiencies consisted 67.2% of patients. Mean follow-up time was 27 months (6 months-5 years). Median neutrophil, lymphocyte, and thrombocyte engraftment days were similar in comparison of both donor type and stem cell source. The most common complication was acute GvHD in 15 (25.8%) patients. In total, five patients (31%) belonging to the SCID group and 10 patients (23.8%) belonging to the non-SCID group died. Our total mortality rate was 15 (25.8%) in all patients. Conclusions We would like to present our HSCT experiences as a pediatric immunology transplantation center. Existing severe infections before transplantation period, BCGitis, and CMV are important issues of transplantation in Turkey. However, the follow-up time is shorter than some studies, our results regarding complications and survival are similar to previous reports.en_US
dc.identifier.citationErol Cipe, F., Adakli Aksoy, B., Aydogdu, S., Dikme, G., Kiykim, A., Aydogmus, C., ... & Fisgin, T. Primary immunodeficiencies: HSCT experiences of a single center in Turkey. Pediatric Transplantation, e14063.en_US
dc.identifier.doi10.1111/petr.14063en_US
dc.identifier.issn1397-3142en_US
dc.identifier.issn1399-3046en_US
dc.identifier.pmid34092004en_US
dc.identifier.scopus2-s2.0-85107196691en_US
dc.identifier.scopusqualityQ2en_US
dc.identifier.urihttps://doi.org/10.1111/petr.14063
dc.identifier.urihttps://hdl.handle.net/20.500.12713/1792
dc.identifier.wosWOS:000658020800001en_US
dc.identifier.wosqualityQ4en_US
dc.indekslendigikaynakWeb of Scienceen_US
dc.indekslendigikaynakScopusen_US
dc.indekslendigikaynakPubMeden_US
dc.institutionauthorErol Çipe, Funda
dc.institutionauthorBozkurt, Ceyhun
dc.language.isoenen_US
dc.publisherWILEYen_US
dc.relation.ispartofPEDIATRIC TRANSPLANTATIONen_US
dc.relation.publicationcategoryMakale - Uluslararası Hakemli Dergi - Kurum Öğretim Elemanıen_US
dc.rightsinfo:eu-repo/semantics/closedAccessen_US
dc.subjectAllogeneic Stem Cell Transplantationen_US
dc.subjectBone Marrow Transplantationen_US
dc.subjectHematopoietic Stem Cell Transplantationen_US
dc.subjectPediatric Transplantationen_US
dc.subjectPrimary Immunodeficiencyen_US
dc.titlePrimary immunodeficiencies: HSCT experiences of a single center in Turkeyen_US
dc.typeArticleen_US

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