Hematopoietic stem cell transplantation from unrelated donors in children with DOCK8 deficiency

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dc.contributor.authorUygun, Dilara Fatma K.
dc.contributor.authorUygun, Vedat
dc.contributor.authorReisli, Ismail
dc.contributor.authorKeles, Sevgi
dc.contributor.authorOzen, Ahmet
dc.contributor.authorYilmaz, Mustafa
dc.contributor.authorSayar, Esra H.
dc.date.accessioned2024-02-23T14:24:37Z
dc.date.available2024-02-23T14:24:37Z
dc.date.issued2017
dc.departmentNEÜen_US
dc.description.abstractDIDS is a unique form of combined immune deficiency characterized by an unusual susceptibility to cutaneous viral infections, severe allergies with eosinophilia and elevated immunoglobulin E titers, autoimmunity, and cancer. HSCT is considered the standard of care for this deadly disease. We have retrospectively analyzed the outcome of allogeneic HSCT from unrelated donors in patients with DIDS. Data from four patients, with five transplants, are presented. All patients received transplants from unrelated donors' BM, except for one patient who received a cord blood transplant. The conditioning regimens were based on myeloablative protocols for BM derived transplants; a NM regimen was pursued for the patient who received a cord blood transplant, which resulted in graft rejection. Although recurrent pneumonia and skin infections resolved immediately after transplantation, all patients subsequently developed human herpesvirus infection, including cutaneous herpetic lesions, cytomegalovirus reactivation, and zona zoster, which could be attributed to the use of ATG. Despite the presence of serious morbidities prior to transplantation, all patients recovered successfully. DIDS can be successfully treated with allogeneic HSCT from unrelated donors following a myeloablative conditioning regimen, with a reasonable safety profile.en_US
dc.identifier.doi10.1111/petr.13015
dc.identifier.issn1397-3142
dc.identifier.issn1399-3046
dc.identifier.issue7en_US
dc.identifier.pmid28664550en_US
dc.identifier.scopus2-s2.0-85021735138en_US
dc.identifier.scopusqualityQ2en_US
dc.identifier.urihttps://doi.org/10.1111/petr.13015
dc.identifier.urihttps://hdl.handle.net/20.500.12452/14031
dc.identifier.volume21en_US
dc.identifier.wosWOS:000412845900009en_US
dc.identifier.wosqualityQ3en_US
dc.indekslendigikaynakWeb of Scienceen_US
dc.indekslendigikaynakScopusen_US
dc.indekslendigikaynakPubMeden_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.subjectDock8 Deficiencyen_US
dc.subjectHscten_US
dc.subjectHyperimmunoglobulin E Syndromeen_US
dc.subjectUnrelated Donoren_US
dc.titleHematopoietic stem cell transplantation from unrelated donors in children with DOCK8 deficiencyen_US
dc.typeArticleen_US

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