Single-Center Study of 72 Patients with Severe Combined Immunodeficiency: Clinical and Laboratory Features and Outcomes

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dc.contributor.authorBayram, Ozlem
dc.contributor.authorHaskologlu, Sule
dc.contributor.authorBayrakoglu, Deniz
dc.contributor.authorBal, Sevgi Kostel
dc.contributor.authorIslamoglu, Candan
dc.contributor.authorCipe, Funda Erol
dc.contributor.authorKendirli, Tanil
dc.date.accessioned2024-02-23T13:56:15Z
dc.date.available2024-02-23T13:56:15Z
dc.date.issued2021
dc.departmentNEÜen_US
dc.description.abstractSevere combined immunodeficiency is an inborn error of immunity characterized by impairments in the numbers and functions of T and B lymphocytes due to various genetic causes, and if it remains untreated, patients succumb to infections during the first 2 years of life. Purpose and Methods This study reported retrospective data from 72 infants diagnosed with SCID including their major clinical features, HSCT characteristics, and outcomes over a 20-year period (1997-2017). Results Sixty-one of 72 SCID patients in the study underwent HSCT from 1997 to 2017. Median ages at the time of diagnosis and transplantation were 3.5 months and 5 months, respectively. Consanguinity was present in 68% of the patients, and T - B - NK + phenotype was predominantly identified. The overall survival was 80.3% over a 20-year period. However, the patients transplanted during an active infection had a lower survival rate of 73.9% compared to 100% for patients transplanted infection-free or with a previous infection that had resolved. The survival rate was significantly higher among recipients of HLA-identical transplants (92.9%), compared to recipients of mismatched related transplants (70%). The overall survival increased from 50 (1997-2006) to 85% (2007-2017) during the last 10 years. Conclusions This is one of the largest single-center studies in Turkey with extensive experience about SCID patients. Early diagnosis of SCID patients before the onset of an infection and early transplantation are shown to be extremely important factors affecting the outcome and increasing the survival regardless of the donor type based on the results of this study.en_US
dc.identifier.doi10.1007/s10875-021-01062-y
dc.identifier.endpage1573en_US
dc.identifier.issn0271-9142
dc.identifier.issn1573-2592
dc.identifier.issue7en_US
dc.identifier.pmid34114123en_US
dc.identifier.scopus2-s2.0-85107734495en_US
dc.identifier.scopusqualityQ1en_US
dc.identifier.startpage1563en_US
dc.identifier.urihttps://doi.org/10.1007/s10875-021-01062-y
dc.identifier.urihttps://hdl.handle.net/20.500.12452/11144
dc.identifier.volume41en_US
dc.identifier.wosWOS:000659780700002en_US
dc.identifier.wosqualityQ1en_US
dc.indekslendigikaynakWeb of Scienceen_US
dc.indekslendigikaynakScopusen_US
dc.indekslendigikaynakPubMeden_US
dc.language.isoenen_US
dc.publisherSpringer/Plenum Publishersen_US
dc.relation.ispartofJournal Of Clinical Immunologyen_US
dc.relation.publicationcategoryMakale - Uluslararası Hakemli Dergi - Kurum Öğretim Elemanıen_US
dc.rightsinfo:eu-repo/semantics/closedAccessen_US
dc.subjectSevere Combined Immunodeficiency (Scid)en_US
dc.subjectHematopoietic Stem Cell Transplantaion (Hsct)en_US
dc.subjectClinical Featuresen_US
dc.subjectOutcomesen_US
dc.titleSingle-Center Study of 72 Patients with Severe Combined Immunodeficiency: Clinical and Laboratory Features and Outcomesen_US
dc.typeArticleen_US

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