{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,28]],"date-time":"2026-07-28T10:19:22Z","timestamp":1785233962526,"version":"3.55.0"},"reference-count":53,"publisher":"Oxford University Press (OUP)","issue":"7","license":[{"start":{"date-parts":[[2015,4,1]],"date-time":"2015-04-01T00:00:00Z","timestamp":1427846400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/pages\/standard-publication-reuse-rights"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2015,4,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:p>The mechanisms underlying thymoma-associated immunodeficiency are largely unknown, and the significance of increased blood \u03b3\u03b4 \u03a4 cells often remains elusive. In this study we address these questions based on an index patient with thymoma, chronic visceral leishmaniasis, myasthenia gravis, and a marked increase of rare \u03b3\u03b4 T cell subsets in the peripheral blood. This patient showed cutaneous anergy, even though he had normal numbers of peripheral blood total lymphocytes as well as CD4+ and CD8+ T cells. Despite his chronic infection, analyses of immunophenotypes and spectratyping of his lymphocytes revealed an unusual accumulation of naive \u03b3\u03b4 and \u03b1\u03b2 T cells, suggesting a generalized T cell activation defect. Functional studies in vitro demonstrated substantially diminished IL-2 and IFN-\u03b3 production following TCR stimulation of his \u201cuntouched\u201d naive CD4+ T cells. Biochemical analysis revealed that his \u03b3\u03b4 and \u03b1\u03b2 T cells carried an altered TCR complex with reduced amounts of the \u03b6-chain (CD247). No mutations were found in the CD247 gene that encodes the homodimeric \u03b6 protein. The diminished presence of CD247 and increased numbers of \u03b3\u03b4 T cells were also observed in thymocyte populations obtained from three other thymoma patients. Thus, our findings describe a novel type of a clinically relevant acquired T cell immunodeficiency in thymoma patients that is distinct from Good\u2019s syndrome. Its characteristics are an accumulation of CD247-deficient, hyporresponsive naive \u03b3\u03b4 and \u03b1\u03b2 T cells and an increased susceptibility to infections.<\/jats:p>","DOI":"10.4049\/jimmunol.1402805","type":"journal-article","created":{"date-parts":[[2015,3,3]],"date-time":"2015-03-03T03:33:11Z","timestamp":1425353591000},"page":"3045-3053","source":"Crossref","is-referenced-by-count":34,"title":["A Novel Thymoma-Associated Immunodeficiency with Increased Naive T Cells and Reduced CD247 Expression"],"prefix":"10.1093","volume":"194","author":[{"given":"Petros","family":"Christopoulos","sequence":"first","affiliation":[{"name":"Department of Pathology, University of Freiburg Medical Center , 79106 Freiburg ,","place":["Germany"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elaine P","family":"Dopfer","sequence":"additional","affiliation":[{"name":"Department of Molecular Immunology, BIOSS Centre for Biological Signalling Studies, Faculty of Biology, University of Freiburg Medical Center , 79106 Freiburg ,","place":["Germany"]},{"name":"Center for Chronic Immunodeficiency CCI, University of Freiburg Medical Center , 79106 Freiburg ,","place":["Germany"]},{"name":"Max-Planck-Institute of Immunobiology and Epigenetics , 79108 Freiburg ,","place":["Germany"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Miroslav","family":"Malkovsky","sequence":"additional","affiliation":[{"name":"Department of Medical Microbiology and Immunology, University of Wisconsin School of Medicine and Public Health , Madison, WI 53726"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Philipp R","family":"Esser","sequence":"additional","affiliation":[{"name":"Department of Dermatology and Venereology (Allergy Research Group), University of Freiburg Medical Center , 79106 Freiburg ,","place":["Germany"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hans-Eckart","family":"Schaefer","sequence":"additional","affiliation":[{"name":"Department of Pathology, University of Freiburg Medical Center , 79106 Freiburg ,","place":["Germany"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alexander","family":"Marx","sequence":"additional","affiliation":[{"name":"Institute of Pathology, University Medical Center Mannheim , 68167 Mannheim ,","place":["Germany"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sylvia","family":"Kock","sequence":"additional","affiliation":[{"name":"Department of Pathology, University of Freiburg Medical Center , 79106 Freiburg ,","place":["Germany"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nicole","family":"Rupp","sequence":"additional","affiliation":[{"name":"Department of Pathology, University of Freiburg Medical Center , 79106 Freiburg ,","place":["Germany"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Myriam R","family":"Lorenz","sequence":"additional","affiliation":[{"name":"Institute for Transfusion Medicine, University Ulm , 89081 Ulm ,","place":["Germany"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Klaus","family":"Schwarz","sequence":"additional","affiliation":[{"name":"Institute for Transfusion Medicine, University Ulm , 89081 Ulm ,","place":["Germany"]},{"name":"Institute for Clinical Transfusion Medicine and Immunogenetics Ulm, German Red Cross Blood Service , Baden-W\u00fcrttemberg\u2013Hessen, 89081 Ulm ,","place":["Germany"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jan","family":"Harder","sequence":"additional","affiliation":[{"name":"Department of Gastroenterology, University of Freiburg Medical Center , 79106 Freiburg ,","place":["Germany"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stefan F","family":"Martin","sequence":"additional","affiliation":[{"name":"Department of Dermatology and Venereology (Allergy Research Group), University of Freiburg Medical Center , 79106 Freiburg ,","place":["Germany"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Martin","family":"Werner","sequence":"additional","affiliation":[{"name":"Department of Pathology, University of Freiburg Medical Center , 79106 Freiburg ,","place":["Germany"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christian","family":"Bogdan","sequence":"additional","affiliation":[{"name":"Institute of Clinical Microbiology, Immunology and Hygiene, Friedrich Alexander University Erlangen\u2013Nuremberg and University Hospital Erlangen , 91054 Erlangen ,","place":["Germany"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wolfgang W A","family":"Schamel","sequence":"additional","affiliation":[{"name":"Department of Molecular Immunology, BIOSS Centre for Biological Signalling Studies, Faculty of Biology, University of Freiburg Medical Center , 79106 Freiburg ,","place":["Germany"]},{"name":"Center for Chronic Immunodeficiency CCI, University of Freiburg Medical Center , 79106 Freiburg ,","place":["Germany"]},{"name":"Max-Planck-Institute of Immunobiology and Epigenetics , 79108 Freiburg ,","place":["Germany"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paul","family":"Fisch","sequence":"additional","affiliation":[{"name":"Department of Pathology, University of Freiburg Medical Center , 79106 Freiburg ,","place":["Germany"]}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2015,4,1]]},"reference":[{"key":"2025030705360695400_r1","first-page":"30","article-title":"A case report of giant cell myocarditis and myositis observed during the clinical course of invasive thymoma associated with myasthenia gravis","volume":"53","author":"Tanahashi","year":"2004","journal-title":"Keio 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