{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,27]],"date-time":"2026-10-27T09:56:28Z","timestamp":1793094988824,"version":"4.5.0"},"reference-count":38,"publisher":"Wiley","issue":"12","license":[{"start":{"date-parts":[[2023,9,20]],"date-time":"2023-09-20T00:00:00Z","timestamp":1695168000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["APMIS"],"published-print":{"date-parts":[[2023,12]]},"abstract":"<jats:p>Good's syndrome, an infrequent adult\u2010onset immunodeficiency is characterized by the triad of thymoma, hypogammaglobulinemia, and increased susceptibility to recurrent infections. The clinical presentation is highly variable, with a spectrum ranging from recurrent bacterial and opportunistic infections to concomitant autoimmune diseases and, sometimes malignant pathologies. Due to heterogeneous clinical phenotypes and the lack of adequate diagnostic criteria, its recognition is often challenging, even delaying it by years. It is one of the most unusual, less studied form of the immune deficiency syndromes with a still unknown pathophysiology. It was initially considered a thymoma\u2010associated variant of primary antibody deficiencies with a reduced or absent number of mature B cells, but it later emerged that significant defects of T cell\u2010mediated immune functions are the underlying cause of opportunistic infections. On the basis of current evidence, Good's syndrome is evaluated as a distinct acquired form of combined immunodeficiency states and classified as a phenocopy of primary immunodeficiency diseases. Epigenetic and acquired genetic factors can play an ultimate role in its evolution.<\/jats:p>","DOI":"10.1111\/apm.13351","type":"journal-article","created":{"date-parts":[[2023,9,20]],"date-time":"2023-09-20T13:43:05Z","timestamp":1695217385000},"page":"698-704","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":21,"title":["Good's syndrome: brief overview of an enigmatic immune deficiency"],"prefix":"10.1111","volume":"131","author":[{"given":"Ferenc","family":"Sipos","sequence":"first","affiliation":[{"name":"Division of Immunology, Department of Internal Medicine and Hematology Semmelweis University  Budapest Hungary"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gy\u00f6rgyi","family":"M\u0171zes","sequence":"additional","affiliation":[{"name":"Division of Immunology, Department of Internal Medicine and Hematology Semmelweis University  Budapest Hungary"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2023,9,20]]},"reference":[{"key":"e_1_2_6_2_1","first-page":"1","article-title":"Agammaglobulinaemia\u2014a provocative experiment of nature","volume":"26","author":"Good RA","year":"1954","journal-title":"Bull Univ Min"},{"key":"e_1_2_6_3_1","doi-asserted-by":"publisher","DOI":"10.1136\/jcp.56.1.12"},{"key":"e_1_2_6_4_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00277-003-0635-z"},{"key":"e_1_2_6_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.clim.2010.01.006"},{"key":"e_1_2_6_6_1","doi-asserted-by":"publisher","DOI":"10.3389\/fimmu.2021.679556"},{"issue":"111","key":"e_1_2_6_7_1","first-page":"528","article-title":"An unexpected diagnosis: Good's syndrome","volume":"78","author":"M\u0171zes G","year":"2023","journal-title":"Allergy"},{"key":"e_1_2_6_8_1","doi-asserted-by":"publisher","DOI":"10.1093\/cid\/civ269"},{"key":"e_1_2_6_9_1","doi-asserted-by":"publisher","DOI":"10.1002\/ijc.11099"},{"key":"e_1_2_6_10_1","doi-asserted-by":"publisher","DOI":"10.1007\/s13244-012-0201-0"},{"key":"e_1_2_6_11_1","doi-asserted-by":"publisher","DOI":"10.1038\/ng.3016"},{"key":"e_1_2_6_12_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ccell.2018.01.003"},{"key":"e_1_2_6_13_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.autrev.2015.09.005"},{"key":"e_1_2_6_14_1","doi-asserted-by":"publisher","DOI":"10.21037\/med.2019.08.03"},{"key":"e_1_2_6_15_1","doi-asserted-by":"publisher","DOI":"10.3389\/fimmu.2012.00019"},{"key":"e_1_2_6_16_1","doi-asserted-by":"publisher","DOI":"10.1038\/cmi.2010.74"},{"key":"e_1_2_6_17_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41590-018-0128-9"},{"key":"e_1_2_6_18_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.imlet.2020.12.010"},{"key":"e_1_2_6_19_1","doi-asserted-by":"publisher","DOI":"10.3389\/fimmu.2022.908453"},{"key":"e_1_2_6_20_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.clim.2016.07.025"},{"key":"e_1_2_6_21_1","doi-asserted-by":"publisher","DOI":"10.1097\/00005792-200103000-00005"},{"key":"e_1_2_6_22_1","doi-asserted-by":"publisher","DOI":"10.1177\/039463201002300129"},{"key":"e_1_2_6_23_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.clim.2018.11.009"},{"key":"e_1_2_6_24_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.aller.2009.07.002"},{"key":"e_1_2_6_25_1","doi-asserted-by":"publisher","DOI":"10.1111\/cei.13216"},{"key":"e_1_2_6_26_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2002-08-2638"},{"key":"e_1_2_6_27_1","doi-asserted-by":"publisher","DOI":"10.1038\/76233"},{"key":"e_1_2_6_28_1","doi-asserted-by":"publisher","DOI":"10.1155\/2018\/6212410"},{"key":"e_1_2_6_29_1","doi-asserted-by":"publisher","DOI":"10.4049\/jimmunol.1402805"},{"key":"e_1_2_6_30_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2010-05-286161"},{"key":"e_1_2_6_31_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.clim.2012.10.006"},{"key":"e_1_2_6_32_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.clim.2014.04.002"},{"key":"e_1_2_6_33_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMc1012485"},{"issue":"1","key":"e_1_2_6_34_1","first-page":"1","article-title":"Primary immunodeficiency diseases. Report of an IUIS scientific committee. International Union of Immunological Societies","volume":"118","year":"1999","journal-title":"Clin Exp Immunol"},{"key":"e_1_2_6_35_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1081-1206(10)61142-8"},{"key":"e_1_2_6_36_1","doi-asserted-by":"publisher","DOI":"10.3389\/fimmu.2021.815710"},{"key":"e_1_2_6_37_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10875-022-01352-z"},{"key":"e_1_2_6_38_1","first-page":"31","article-title":"Primary hypogammaglobulinaemia: a survey of clinical manifestations and complications","volume":"86","author":"Hermaszewski RA","year":"1993","journal-title":"Q J Med"},{"key":"e_1_2_6_39_1","doi-asserted-by":"publisher","DOI":"10.3389\/fonc.2014.00307"}],"container-title":["APMIS"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/apm.13351","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,11,6]],"date-time":"2023-11-06T10:55:41Z","timestamp":1699268141000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/apm.13351"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,9,20]]},"references-count":38,"journal-issue":{"issue":"12","published-print":{"date-parts":[[2023,12]]}},"alternative-id":["10.1111\/apm.13351"],"URL":"https:\/\/doi.org\/10.1111\/apm.13351","archive":["Portico"],"relation":{},"ISSN":["0903-4641","1600-0463"],"issn-type":[{"value":"0903-4641","type":"print"},{"value":"1600-0463","type":"electronic"}],"subject":[],"published":{"date-parts":[[2023,9,20]]},"assertion":[{"value":"2023-08-25","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2023-09-05","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2023-09-20","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}