Autoimmune Cytopenias Are Highly Associated with Inborn Errors of Immunity and They May Be the Initial Presentations in Cases without Severe Infections

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dc.authoridTOPYILDIZ, Ezgi/0000-0002-9260-8157
dc.authoridTASKIN, RAZIYE BURCU/0000-0003-1734-8102
dc.contributor.authorTaskin, Raziye Burcu
dc.contributor.authorTopyildiz, Ezgi
dc.contributor.authorKaraca, Neslihan Edeer
dc.contributor.authorAksu, Guzide
dc.contributor.authorKarapinar, Deniz Yilmaz
dc.contributor.authorKutukculer, Necil
dc.date.accessioned2024-08-31T07:50:11Z
dc.date.available2024-08-31T07:50:11Z
dc.date.issued2024
dc.departmentEge Üniversitesien_US
dc.description.abstractIntroduction: Inborn errors of immunity (IEIs) are inherited disorders that present with increased susceptibility to infections as well as noninfectious complications. Due to the aberrant immune functions of patients with IEI, autoimmune cytopenia (AIC) may be the initial finding, which makes diagnosis a challenge. We aimed to evaluate the clinical course, laboratory findings, and treatment response of AIC in children with IEI. Methods: Data of children with autoimmune hemolytic anemia (AIHA) and/or immune thrombocytopenic purpura (ITP) were obtained from a retrospective chart review of IEI patients diagnosed and followed in our center. Demographic and clinical features and therapeutic outcomes were evaluated. Immunologic findings were compared between patients with AIHA, ITP, and Evans syndrome (ES). The patients were also divided into two subgroups based on the presence or absence of immune dysregulation diseases (IDDs), and all data were compared between these two groups. Results: Out of 562 patients with IEI, 6% (n: 34) had AIC which were ITP (23.5%), AIHA (35.5%), and ES (41.2%). AIC was the initial finding in 50% of these 34 patients. Patients with ES had a higher mean percentage of CD8+ T lymphocytes than ITP patients (40.77 +/- 20.21% vs. 22.33 +/- 12.48%, p = 0.011). Patients with IDDs were more likely to develop ES (p = 0.004), lymphoproliferation (p = 0.005), and resistance to first-line therapy (p = 0.021) than other IEI groups. Conclusion: This study shows that AIC may be the initial finding of IEI, particularly when lymphoproliferation and resistance to first-line therapy co-occur. Therefore, detailed investigation should be offered to all patients to avoid diagnostic delay.en_US
dc.identifier.doi10.1159/000535258
dc.identifier.endpage401en_US
dc.identifier.issn1018-2438
dc.identifier.issn1423-0097
dc.identifier.issue4en_US
dc.identifier.pmid38154455en_US
dc.identifier.scopus2-s2.0-85189462073en_US
dc.identifier.scopusqualityQ2en_US
dc.identifier.startpage392en_US
dc.identifier.urihttps://doi.org/10.1159/000535258
dc.identifier.urihttps://hdl.handle.net/11454/105127
dc.identifier.volume185en_US
dc.identifier.wosWOS:001136420500001en_US
dc.identifier.wosqualityQ3en_US
dc.indekslendigikaynakWeb of Scienceen_US
dc.indekslendigikaynakScopusen_US
dc.indekslendigikaynakPubMeden_US
dc.language.isoenen_US
dc.publisherKargeren_US
dc.relation.ispartofInternational Archives of Allergy and Immunologyen_US
dc.relation.publicationcategoryMakale - Uluslararası Hakemli Dergi - Kurum Öğretim Elemanıen_US
dc.rightsinfo:eu-repo/semantics/closedAccessen_US
dc.snmz20240831_Uen_US
dc.subjectPrimary Immunodeficiencyen_US
dc.subjectAutoimmunityen_US
dc.subjectImmune Dysregulationen_US
dc.subjectCytopeniaen_US
dc.titleAutoimmune Cytopenias Are Highly Associated with Inborn Errors of Immunity and They May Be the Initial Presentations in Cases without Severe Infectionsen_US
dc.typeArticleen_US

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