Hematopoietic stem cell transplantation in CD40 ligand deficiency: a single-center experience

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dc.authoridGülsun Tezcan Karasu / 0000-0001-5700-5919
dc.authoridVedat Uygun / 0000-0003-3257-7798
dc.authorscopusidGülsun Tezcan Karasu / 56382958800
dc.authorscopusidVedat Uygun / 10043117000
dc.authorwosidGülsun Tezcan Karasu / IAF-9902-2023
dc.authorwosidVedat Uygun / AGH-4534-2022
dc.contributor.authorUygun, Dilara Fatma Kocacık
dc.contributor.authorUygun, Vedat
dc.contributor.authorTezcan Karasu, Gülsun
dc.contributor.authorDaloğlu, Hayriye
dc.contributor.authorÖztürkmen, Seda Irmak
dc.contributor.authorÇelmeli, Fatih
dc.contributor.authorTorun, Selda Hançerli
dc.contributor.authorÖzen, Ahmet
dc.contributor.authorBarış, Safa
dc.contributor.authorAydıner, Elif Karakoç
dc.contributor.authorYalçın, Koray
dc.contributor.authorKılıç, Suar Çaki
dc.contributor.authorHazar, Volkan
dc.contributor.authorBingöl, Aysen
dc.contributor.authorYeşilipek, Akif
dc.date.accessioned2020-08-30T20:06:08Z
dc.date.available2020-08-30T20:06:08Z
dc.date.issued2020
dc.departmentİstinye Üniversitesi, Tıp Fakültesi, Dahili Tıp Bilimleri Bölümüen_US
dc.descriptionUygun, Vedat ; Karasu, Gulsun Tezcan (isu author)en_US
dc.description.abstractDeficiency of the CD40L, expressed on the surface of T lymphocytes, is caused by mutations in the glycoproteinCD40L (CD154)gene. Resulting defective humoral and cellular responses cause a clinical presentation that includes recurrent sinopulmonary bacterial infections, opportunistic infections, sclerosing cholangitis, neutropenia, and autoimmune manifestations. HSCT represents the only curative treatment modality. However, the therapeutic decision to use HSCT proves challenging in many cases, mainly due to the lack of a phenotype-genotype correlation. We retrospectively reviewed patients with CD40L deficiency who were transplanted in Antalya and Goztepe MedicalPark Pediatric HSCT units from 2014 to 2019 and followed by Akdeniz University School of Medicine Department of Pediatric Immunology. The records of eight male cases, including one set of twins, were evaluated retrospectively. As two transplants each were performed on the twins, a total of ten transplants were evaluated. Conditioning regimens were predominantly based on myeloablative protocols, except for the twins, who received a non-myeloablative regimen for their first transplantation. Median neutrophil and platelet engraftment days were 13 (range 10-19) and 14 (range 10-42) days, respectively. In seven of ten transplants, a CMV reactivation was developed without morbidity. None of the patients developed GVHD, except for one mild case of acute GVHD. All patients survived, and the median follow-up was 852 days. Our data show that HSCT for patients with CD40 ligand deficiency is a potentially effective treatment for long-term disease control.en_US
dc.identifier.citationUygun, D. F. K., Uygun, V., Karasu, G. T., Daloglu, H., Ozturkmen, S. I., Celmeli, F., … Yesilipek, A. (n.d.). Hematopoietic stem cell transplantation in CD40 ligand deficiency: A single-center experience. PEDIATRIC TRANSPLANTATION. https://doi.org/10.1111/petr.13768en_US
dc.identifier.doi10.1111/petr.13768en_US
dc.identifier.issn1397-3142en_US
dc.identifier.issn1399-3046en_US
dc.identifier.pmid32573870en_US
dc.identifier.scopus2-s2.0-85087307425en_US
dc.identifier.scopusqualityQ2en_US
dc.identifier.urihttps://doi.org/10.1111/petr.13768
dc.identifier.urihttps://hdl.handle.net/20.500.12713/384
dc.identifier.wosWOS:000541941200001en_US
dc.identifier.wosqualityQ4en_US
dc.indekslendigikaynakWeb of Scienceen_US
dc.indekslendigikaynakScopusen_US
dc.indekslendigikaynakPubMeden_US
dc.institutionauthorUygun, Vedaten_US
dc.institutionauthorTezcan Karasu, Gülsünen_US
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.subjectCd40l Deficiencyen_US
dc.subjectChildrenen_US
dc.subjectHematopoietic Stem Cell Transplantationen_US
dc.titleHematopoietic stem cell transplantation in CD40 ligand deficiency: a single-center experienceen_US
dc.typeArticleen_US

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