{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,2]],"date-time":"2026-08-02T16:44:54Z","timestamp":1785689094896,"version":"3.56.0"},"reference-count":29,"publisher":"SAGE Publications","license":[{"start":{"date-parts":[[2023,5,1]],"date-time":"2023-05-01T00:00:00Z","timestamp":1682899200000},"content-version":"unspecified","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"},{"start":{"date-parts":[[2023,5,1]],"date-time":"2023-05-01T00:00:00Z","timestamp":1682899200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/journals.sagepub.com\/page\/policies\/text-and-data-mining-license"}],"content-domain":{"domain":["journals.sagepub.com"],"crossmark-restriction":true},"short-container-title":["Therapeutic Advances in Allergy and Rhinology"],"published-print":{"date-parts":[[2023,5]]},"abstract":"<jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Selective anti-polysaccharide antibody deficiency (SPAD) with CD5 B-cell predominance and autoimmune phenomena was identified in a male cohort first reported by Antall et al in 1999. The phenotypically likewise and genotypically identical X-linked immunodeficiency with magnesium defect, Epstein\u2013Barr Virus infection, and neoplasia (XMEN) disease was defined as a novel primary immunodeficiency (PID) in 2011. Recent studies of the magnesium transporter 1 (MAGT1) gene mutation reveal glycosylation defects contributing to more phenotypic variance than the \u201cXMEN\u201d title pathologies. The updated title, \u201cX-linked MAGT1 deficiency with increased susceptibility to EBV-infection and N-linked glycosylation defect,\u201d was proposed in 2020.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Objectives<\/jats:title>\n                    <jats:p>To reflect the patient population more accurately, a prospective classification update may consider MAGT1 glycobiological errors contributing to phenotypic variance but also pre-genetic testing era reports with CD5 B-cell predominance.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>Patient 1 from Antall et al presented at 28 years of age for further immunological evaluation of his CD5\/CD19 B-cell predominance diagnosed at 5 years old.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Design<\/jats:title>\n                    <jats:p>Immune re-evaluation done through flow cytometry and next-generation sequencing.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>Flow cytometry B-cell phenotyping revealed persistent CD5+CD19+ (93%). Flow cytometric histogram quantified reduced activator CD16+CD56+ natural killer and CD8+ T-cell receptor, Group 2, Member D (NKG2D) glycoprotein expression. A c.923-1_934 deletion loss of function mutation was identified in the MAGT1 gene.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>We suggest the novel PID XMEN, based on its CD5 B-cell predominance, had been discovered and reported over a decade earlier as CD5+ PID based on the MAGT1 mutation found in the same. We encourage consideration of combining these labels and recent findings to offer the most accurate classification of this disease.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1177\/27534030231199675","type":"journal-article","created":{"date-parts":[[2023,9,11]],"date-time":"2023-09-11T04:07:50Z","timestamp":1694405270000},"update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":3,"title":["CD5 B-Cell Predominant Primary Immunodeficiency: Part of the Spectrum of\n                    <i>MAGT1<\/i>\n                    Deficiency"],"prefix":"10.1177","volume":"14","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-6500-8279","authenticated-orcid":false,"given":"Marija J.","family":"Rowane","sequence":"first","affiliation":[{"name":"Children's Hospital of St. Francis at Oklahoma State University, Tulsa, Oklahoma"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Benjamin C.","family":"Stewart-Bates","sequence":"additional","affiliation":[{"name":"Wake Forest Baptist Medical Center, Winston-Salem, North Carolina"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rayna J.","family":"Doll","sequence":"additional","affiliation":[{"name":"Spokane Allergy &amp; Asthma Clinic, Spokane, Washington"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Howard J.","family":"Meyerson","sequence":"additional","affiliation":[{"name":"Division of Clinical Pathology, Case Western Reserve University School of Medicine, Cleveland, Ohio"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"suffix":"III","given":"John S.","family":"Venglarcik","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Northeast Ohio Medical University, Rootstown, Ohio"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7015-4561","authenticated-orcid":false,"given":"Meghan","family":"Callahan","sequence":"additional","affiliation":[{"name":"Lake Erie College of Osteopathic Medicine, Erie, Pennsylvania"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lauren","family":"Fill","sequence":"additional","affiliation":[{"name":"Allergy\/Immunology Associates, Inc., Mayfield Heights, Ohio"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Remie","family":"Saab","sequence":"additional","affiliation":[{"name":"University Hospitals Community Consortium Geauga, Geauga, Ohio"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hans D.","family":"Ochs","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, University of Washington School of Medicine, Seattle, Washington"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"suffix":"Jr.","given":"Robert W.","family":"Hostoffer","sequence":"additional","affiliation":[{"name":"Allergy\/Immunology Associates, Inc., Mayfield Heights, Ohio"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"179","published-online":{"date-parts":[[2023,9,11]]},"reference":[{"key":"e_1_3_4_2_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaci.2015.04.049"},{"key":"e_1_3_4_3_2","doi-asserted-by":"publisher","DOI":"10.1007\/s10875-020-00758-x"},{"key":"e_1_3_4_4_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0091-6749(99)70236-8"},{"key":"e_1_3_4_5_2","volume-title":"Recent Research Developments in Allergy and Clinical Immunology","author":"Hostoffer R","year":"2000","unstructured":"Hostoffer R. 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Lippincott Williams & Wilkins; 2000:3\u201332."},{"key":"e_1_3_4_8_2","doi-asserted-by":"publisher","DOI":"10.1684\/mrh.2011.0286"},{"key":"e_1_3_4_9_2","doi-asserted-by":"publisher","DOI":"10.1007\/s10875-020-00790-x"},{"key":"e_1_3_4_10_2","doi-asserted-by":"publisher","DOI":"10.1172\/JCI131116"},{"key":"e_1_3_4_11_2","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.1817815116"},{"key":"e_1_3_4_12_2","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.RA119.008903"},{"key":"e_1_3_4_13_2","doi-asserted-by":"publisher","DOI":"10.1172\/JCI106756"},{"key":"e_1_3_4_14_2","first-page":"289","volume-title":"Immonological Disorders in Infants & Children","author":"Stiehm ER","year":"2004","unstructured":"Stiehm ER, Ochs HD, Winkelstein JA. Chapter 12: immunodeficiency disorders: general considerations. In: Immonological Disorders in Infants & Children, 5th ed. 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