{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,18]],"date-time":"2026-07-18T09:26:08Z","timestamp":1784366768179,"version":"3.55.0"},"reference-count":24,"publisher":"Walter de Gruyter GmbH","issue":"1","license":[{"start":{"date-parts":[[2021,1,1]],"date-time":"2021-01-01T00:00:00Z","timestamp":1609459200000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2021,4,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:sec>\n                  <jats:title>Background<\/jats:title>\n                  <jats:p>Good\u2019s syndrome (GS) is an immunodeficiency disease, causing thymoma, low or absent B-cells, hypogammaglobulinemia, and defects in cell-mediated immunity. The most common clinical presentation is recurrent infection, followed by refractory diarrhea, due to the immunodeficiency. However, there are only few reports on intestinal endoscopy and pathology.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Case summary<\/jats:title>\n                  <jats:p>We report here two typical GS cases with diarrhea as the prominent manifestation. Both cases presented with thymoma combined with immunodeficiency, characterized by hypogammaglobulinemia, low or absent B lymphocytes, and decreased T-cells with inverted CD4<jats:sup>+<\/jats:sup>\/CD8<jats:sup>+<\/jats:sup> T-cell ratio, while two GS patients were evaluated by endoscopy revealed mucosal edema and fine-granular or nodular appearance changes in the small intestine. Histological examination showed chronic inflammation and villous atrophy. A very interesting finding is that the inflammatory cell infiltration in the two GS cases was different. In one case, predominantly CD138<jats:sup>+<\/jats:sup> plasma cells with only scattered CD3<jats:sup>+<\/jats:sup> T-cells infiltration were revealed, while in another, it showed predominantly T-cells infiltration without plasma cells in the lamina propria. Although GS cases shared various clinical characteristics with common variable immunodeficiency (CVID) cases, they still differed from CVID cases in terms of its late onset, lack of familial clusters, low or absent peripheral blood B lymphocytes, absence of lymphoid hyperplasia, and plasma cells infiltration in the lamina propria in some patients. Although both patients had been diagnosed previously with recurrent diarrhea, respiratory infection, and thymoma, the association between these conditions and the possibility of GS was not recognized. The patients had remained misdiagnosed for 2 and 4 years, respectively, even after receiving the diagnosis of thymoma. The rarity of GS was likely the primary cause for the lack of disease recognition. Reporting of these cases will help to alert clinicians and raise awareness of this disease.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Conclusion<\/jats:title>\n                  <jats:p>GS should be considered among the differential diagnoses for patients with unexplained recurrent diarrhea and opportunistic infection. Although it was regarded as a subset of CVID with thymoma, GS had a different clinical-pathological feature from CVID.<\/jats:p>\n               <\/jats:sec>","DOI":"10.1515\/med-2021-0256","type":"journal-article","created":{"date-parts":[[2021,4,1]],"date-time":"2021-04-01T20:46:52Z","timestamp":1617310012000},"page":"532-539","source":"Crossref","is-referenced-by-count":5,"title":["Clinicopathologic features of Good\u2019s syndrome: Two cases and literature review"],"prefix":"10.1515","volume":"16","author":[{"given":"Yi-Dan","family":"Chen","sequence":"first","affiliation":[{"name":"Department of Rheumatology and Immunology, West China Hospital, Sichuan University , Chengdu , Sichuan , People\u2019s Republic of China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zhong-Hui","family":"Wen","sequence":"additional","affiliation":[{"name":"Department of Gastroenterology, West China Hospital, Sichuan University , Chengdu , Sichuan , People\u2019s Republic of China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bing","family":"Wei","sequence":"additional","affiliation":[{"name":"Department of Pathology, West China Hospital, Sichuan University , Chengdu , Sichuan , People\u2019s Republic of China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shu-Yuan","family":"Xiao","sequence":"additional","affiliation":[{"name":"Department of Pathology, The University of Chicago , Chicago , IL , United States of America"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yu-Fang","family":"Wang","sequence":"additional","affiliation":[{"name":"Department of Gastroenterology, West China Hospital, Sichuan University , Chengdu , Sichuan , People\u2019s Republic of China"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"374","published-online":{"date-parts":[[2021,4,1]]},"reference":[{"key":"2022091918210259675_j_med-2021-0256_ref_001","unstructured":"Good RA, Varco RL. A clinical and experimental study of agammaglobulinemia. J Lancet. 1955;75(6):245\u201371. 10.1016\/j.bbabio.2012.04.004."},{"key":"2022091918210259675_j_med-2021-0256_ref_002","doi-asserted-by":"crossref","unstructured":"International Union of Immunological Societies. Primary immunodeficiency diseases. Report of an IUIS scientific committee. Clin Exp Immunol. 1999;118(Supp 1):1\u201328. 10.1046\/j.1365-2249.1999.00109.x.","DOI":"10.1046\/j.1365-2249.1999.00109.x"},{"key":"2022091918210259675_j_med-2021-0256_ref_003","doi-asserted-by":"crossref","unstructured":"Zaman M, Huissoon A, Buckland M, Patel S, Alachkar H, Edgar JD, et al. Clinical and laboratory features of seventy-eight UK patients with good\u2019s syndrome (thymoma and hypogammaglobulinaemia). Clin Exp Immunol. 2019;1:132. 10.1111\/cei.13216.","DOI":"10.1111\/cei.13216"},{"key":"2022091918210259675_j_med-2021-0256_ref_004","doi-asserted-by":"crossref","unstructured":"Kelesidis T, Yang O. Good\u2019s syndrome remains a mystery after 55 years: a systematic review of the scientific evidence. Clin Immunol. 2010;135(3):347\u201363. 10.1016\/j.clim.2010.01.006.","DOI":"10.1016\/j.clim.2010.01.006"},{"key":"2022091918210259675_j_med-2021-0256_ref_005","doi-asserted-by":"crossref","unstructured":"Thomas CR, Wright CD, Loehrer PJ. Thymoma: state of the art. J Clin Oncol. 1999;17(7):2280\u20139. 10.1200\/JCO.1999.17.7.2280.","DOI":"10.1200\/JCO.1999.17.7.2280"},{"key":"2022091918210259675_j_med-2021-0256_ref_006","doi-asserted-by":"crossref","unstructured":"Bernard C, Frih H, Pasquet F, Kerever S, Jamilloux Y, Tronc F, et al. Thymoma associated with autoimmune diseases: 85 cases and literature review. Autoimmun Rev. 2016;15(1):82\u201392. 10.1016\/j.autrev.2015.09.005.","DOI":"10.1016\/j.autrev.2015.09.005"},{"key":"2022091918210259675_j_med-2021-0256_ref_007","doi-asserted-by":"crossref","unstructured":"Sasson SC, Davies S, Chan R, Davies L, Garsia R. Cerebral toxoplasmosis in a patient with myasthenia gravis and thymoma with immunodeficiency\/Good\u2019s syndrome: a case report. BMC Infect Dis. 2016;16(1):457. 10.1186\/s12879-016-1801-y.","DOI":"10.1186\/s12879-016-1801-y"},{"key":"2022091918210259675_j_med-2021-0256_ref_008","doi-asserted-by":"crossref","unstructured":"Kelesidis T, Yang O. Good\u2019s syndrome remains a mystery after 55 years: a systematic review of the scientific evidence. Clin Immunol. 2010;135(3):347\u201363. 10.1016\/j.clim.2010.01.006.","DOI":"10.1016\/j.clim.2010.01.006"},{"key":"2022091918210259675_j_med-2021-0256_ref_009","doi-asserted-by":"crossref","unstructured":"Miyakis S, Pefanis A, Passam FH, Christodulakis GR, Roussou PA, Mountokalakis TD. Thymoma with immunodeficiency (Good\u2019s syndrome): review of the literature apropos three cases. Scand J Infect Dis. 2006;38(4):314\u20139. 10.1080\/00365540500372663.","DOI":"10.1080\/00365540500372663"},{"key":"2022091918210259675_j_med-2021-0256_ref_010","doi-asserted-by":"crossref","unstructured":"Kelleher P, Misbah SA. What is Good\u2019s syndrome? Immunological abnormalities in patients with thymoma. J Clin Pathol. 2003;56(1):12\u20136. 10.1136\/jcp.56.1.12.","DOI":"10.1136\/jcp.56.1.12"},{"key":"2022091918210259675_j_med-2021-0256_ref_011","doi-asserted-by":"crossref","unstructured":"Watts RG, Kelly DR. Fatal varicella infection in a child associated with thymoma and immunodeficiency (Good\u2019s syndrome). Med Pediatr Oncol. 1990;18(3):246\u201351. 10.1002\/mpo.2950180318.","DOI":"10.1002\/mpo.2950180318"},{"key":"2022091918210259675_j_med-2021-0256_ref_012","doi-asserted-by":"crossref","unstructured":"Dong JP, Gao W, Teng GG, Tian Y, Wang HH. Characteristics of Good\u2019s syndrome in China: a systematic review. Chin Med J (Engl). 2017;130(13):1604\u20139. 10.4103\/0366-6999.208234.","DOI":"10.4103\/0366-6999.208234"},{"key":"2022091918210259675_j_med-2021-0256_ref_013","doi-asserted-by":"crossref","unstructured":"Yildiz O, Ozguroglu M, Turna H, Yanmaz T, Kaynak K, Akman C, et al. Thymoma with chronic diarrhea: report of two cases and review of the literature. Med Oncol. 2007;24(1):119\u201323. 10.1007\/BF02685913.","DOI":"10.1007\/BF02685913"},{"key":"2022091918210259675_j_med-2021-0256_ref_014","doi-asserted-by":"crossref","unstructured":"Mancuso A, Gentiluomo M, Vangeli M, Torre MD, Belli LS. Diarrhea as sole presentation of Good\u2019s syndrome mimicking Crohn\u2019s disease. Clin Immunol. 2013;147(1):9\u201310. 10.1016\/j.clim.2013.01.012.","DOI":"10.1016\/j.clim.2013.01.012"},{"key":"2022091918210259675_j_med-2021-0256_ref_015","unstructured":"Gupta S, Saverymuttu SH, Gibbs JS, Evans DJ, Hodgson HJ. Watery diarrhea in a patient with myasthenia gravis, thymoma, and immunodeficiency. Am J Gastroenterol. 1985;80(11):877\u201381."},{"key":"2022091918210259675_j_med-2021-0256_ref_016","doi-asserted-by":"crossref","unstructured":"Salzer U, Warnatz K, Peter HH. Common variable immunodeficiency: an update. Arthritis Res Ther. 2012;14(5):223. 10.1186\/ar4032.","DOI":"10.1186\/ar4032"},{"key":"2022091918210259675_j_med-2021-0256_ref_017","doi-asserted-by":"crossref","unstructured":"Hughes WS, Cerda JJ, Holtzapple P, Brooks FP. Primary hypogammaglobulinemia and malabsorption. Ann Intern Med. 1971;74(6):903\u201310. 10.7326\/0003-4819-74-6-903.","DOI":"10.7326\/0003-4819-74-6-903"},{"key":"2022091918210259675_j_med-2021-0256_ref_018","doi-asserted-by":"crossref","unstructured":"Verne GN, Amann ST, Cosgrove C, Cerda JJ. Chronic diarrhea associated with thymoma and hypogammaglobulinemia (Good\u2019s syndrome). South Med J. 1997;90(4):444\u20136. 10.1097\/00007611-199704000-00018.","DOI":"10.1097\/00007611-199704000-00018"},{"key":"2022091918210259675_j_med-2021-0256_ref_019","doi-asserted-by":"crossref","unstructured":"Rosenow EC, Hurley BT. Disorders of the thymus. Arch Intern Med. 1984;144(4):763\u201370. 10.1001\/archinte.1984.00350160117020.","DOI":"10.1001\/archinte.144.4.763"},{"key":"2022091918210259675_j_med-2021-0256_ref_020","doi-asserted-by":"crossref","unstructured":"Furukawa R, Yanagiya M, Matsumoto J, Hashimoto H, Horiuchi H, Usui K. Good\u2019s syndrome with clinical manifestation after thymectomy: a case report. Respir Med Case Rep. 2018;24:89\u201391. 10.1016\/j.rmcr.2018.04.014.","DOI":"10.1016\/j.rmcr.2018.04.014"},{"key":"2022091918210259675_j_med-2021-0256_ref_021","doi-asserted-by":"crossref","unstructured":"Thuraisingam A. An intrathoracic mass behind severe immunodeficiency: a case report of Good\u2019s syndrome and large type AB thymoma. J Surg Case Rep. 2020;2020(1):rjz394. 10.1093\/jscr\/rjz394.","DOI":"10.1093\/jscr\/rjz394"},{"key":"2022091918210259675_j_med-2021-0256_ref_022","unstructured":"Sena A, Ferreira R, Gon\u00e7alves J, Nobre \u00c2. Surgical treatment of an advanced stage thymoma in a Good\u2019s syndrome patient \u2013 case report. Rev Port Cir Cardiotorac Vasc. 2019;26(4):269\u201371."},{"key":"2022091918210259675_j_med-2021-0256_ref_023","doi-asserted-by":"crossref","unstructured":"DeBoard ZM, Taylor BJ. Good\u2019s syndrome: successful management of thymoma with hypoimmunoglobulinemia. Ann Thorac Surg. 2015;100(5):1903\u20135. 10.1016\/j.athoracsur.2014.12.108.","DOI":"10.1016\/j.athoracsur.2014.12.108"},{"key":"2022091918210259675_j_med-2021-0256_ref_024","doi-asserted-by":"crossref","unstructured":"Jagessar SAR, Long C, Cui B, Zhang F. Improvement of Good\u2019s syndrome by fecal microbiota transplantation: the first case report. J Int Med Res. 2019;47(7):3408\u201315. 10.1177\/0300060519854913.","DOI":"10.1177\/0300060519854913"}],"container-title":["Open Medicine"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.degruyter.com\/document\/doi\/10.1515\/med-2021-0256\/xml","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/www.degruyter.com\/document\/doi\/10.1515\/med-2021-0256\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2022,9,19]],"date-time":"2022-09-19T18:33:27Z","timestamp":1663612407000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.degruyter.com\/document\/doi\/10.1515\/med-2021-0256\/html"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,1,1]]},"references-count":24,"journal-issue":{"issue":"1","published-online":{"date-parts":[[2021,5,14]]},"published-print":{"date-parts":[[2021,5,14]]}},"alternative-id":["10.1515\/med-2021-0256"],"URL":"https:\/\/doi.org\/10.1515\/med-2021-0256","relation":{},"ISSN":["2391-5463"],"issn-type":[{"value":"2391-5463","type":"electronic"}],"subject":[],"published":{"date-parts":[[2021,1,1]]}}}