{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,24]],"date-time":"2026-09-24T23:11:03Z","timestamp":1790291463822,"version":"4.1.0"},"reference-count":21,"publisher":"Wiley","issue":"11","license":[{"start":{"date-parts":[[2017,5,30]],"date-time":"2017-05-30T00:00:00Z","timestamp":1496102400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/100000050","name":"National Heart, Lung, and Blood Institute","doi-asserted-by":"publisher","award":["R01 HL108102"],"award-info":[{"award-number":["R01 HL108102"]}],"id":[{"id":"10.13039\/100000050","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Pediatric Blood &amp; Cancer"],"published-print":{"date-parts":[[2017,11]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Fanconi anemia (FA) is a rare genetic disorder characterized by genome instability, bone marrow failure, and cancer predisposition. Previously, small studies have reported heterogeneous immune dysfunction in FA.<\/jats:p><\/jats:sec><jats:sec><jats:title>Procedure<\/jats:title><jats:p>We performed a detailed immunologic assessment in a large FA cohort who have not undergone bone marrow transplantation or developed malignancies. Comprehensive quantitative and functional immunologic assessment of 29 FA individuals was compared to healthy age\u2010matched controls.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Compared to non\u2010FA persons of similar ages, FA individuals showed lower absolute total B cells (<jats:italic>P<\/jats:italic> &lt; 0.001), lower memory B cells (<jats:italic>P<\/jats:italic> &lt; 0.001), and decreased IgM (<jats:italic>P<\/jats:italic> &lt; 0.001) but normal IgG. NK cells (<jats:italic>P<\/jats:italic> &lt; 0.001) and NK cytotoxicity (<jats:italic>P<\/jats:italic> &lt; 0.001) were decreased. CD4<jats:sup>+<\/jats:sup> T cells were decreased (<jats:italic>P<\/jats:italic> = 0.022), while CD8<jats:sup>+<\/jats:sup> T cell and absolute T\u2010cell numbers were comparable. Cytotoxic T cells (<jats:italic>P<\/jats:italic> &lt; 0.003), and antigen proliferation response to tetanus (<jats:italic>P<\/jats:italic> = 0.019) and candida (<jats:italic>P<\/jats:italic> = 0.019), were diminished in FA. Phytohemagglutinin responses and plasma cytokines were normal. Within FA subjects, adults and older children (\u226510 years) exhibited higher CD8<jats:sup>+<\/jats:sup> T cells than younger children (<jats:italic>P<\/jats:italic> = 0.004). Documented atypical infections were infrequent, although oral human papilloma virus (HPV) prevalence was higher (31% positive) in FA.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Overall, these results demonstrate a high rate of significant humoral and cellular immune dysfunction. Continued longitudinal study of immune function is critical to understand evolution with age, bone marrow failure, and cancer development.<\/jats:p><\/jats:sec>","DOI":"10.1002\/pbc.26599","type":"journal-article","created":{"date-parts":[[2017,5,30]],"date-time":"2017-05-30T05:58:51Z","timestamp":1496123931000},"source":"Crossref","is-referenced-by-count":32,"title":["Impaired immune function in children and adults with Fanconi anemia"],"prefix":"10.1002","volume":"64","author":[{"given":"Kasiani C.","family":"Myers","sequence":"first","affiliation":[{"name":"Division of Bone Marrow Transplantation and Immune Deficiency Cincinnati Children's Hospital Medical Center  Cincinnati Ohio"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sharon","family":"Sauter","sequence":"additional","affiliation":[{"name":"Asthma Research Cincinnati Children's Hospital Medical Center  Cincinnati 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