{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,27]],"date-time":"2026-10-27T09:56:33Z","timestamp":1793094993662,"version":"4.5.0"},"reference-count":23,"publisher":"Oxford University Press (OUP)","issue":"1","license":[{"start":{"date-parts":[[2021,11,16]],"date-time":"2021-11-16T00:00:00Z","timestamp":1637020800000},"content-version":"vor","delay-in-days":1122,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"funder":[{"DOI":"10.13039\/100009250","name":"National Institute of Health Research","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100009250","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100005622","name":"Health Research","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100005622","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2018,12,19]]},"abstract":"<jats:title>Summary<\/jats:title>\n               <jats:p>Good\u2019s syndrome (thymoma and hypogammaglobulinaemia) is a rare secondary immunodeficiency disease, previously reported in the published literature as mainly individual cases or small case series. We use the national UK-Primary Immune Deficiency (UKPID) registry to identify a large cohort of patients in the UK with this PID to review its clinical course, natural history and prognosis. Clinical information, laboratory data, treatment and outcome were collated and analysed. Seventy-eight patients with a median age of 64 years, 59% of whom were female, were reviewed. Median age of presentation was 54 years. Absolute B cell numbers and serum immunoglobulins were very low in all patients and all received immunoglobulin replacement therapy. All patients had undergone thymectomy and nine (12%) had thymic carcinoma (four locally invasive and five had disseminated disease) requiring adjuvant radiotherapy and\/or chemotherapy. CD4 T cells were significantly lower in these patients with malignant thymoma. Seventy-four (95%) presented with infections, 35 (45%) had bronchiectasis, seven (9%) chronic sinusitis, but only eight (10%) had serious invasive fungal or viral infections. Patients with AB-type thymomas were more likely to have bronchiectasis. Twenty (26%) suffered from autoimmune diseases (pure red cell aplasia, hypothyroidism, arthritis, myasthenia gravis, systemic lupus erythematosus, Sj\u00f6gren\u2019s syndrome). There was no association between thymoma type and autoimmunity. Seven (9%) patients had died. Good\u2019s syndrome is associated with significant morbidity relating to infectious and autoimmune complications. Prospective studies are required to understand why some patients with thymoma develop persistent hypogammaglobulinaemia.<\/jats:p>","DOI":"10.1111\/cei.13216","type":"journal-article","created":{"date-parts":[[2018,9,16]],"date-time":"2018-09-16T03:07:51Z","timestamp":1537067271000},"page":"132-138","source":"Crossref","is-referenced-by-count":54,"title":["Clinical and laboratory features of seventy-eight UK patients with Good\u2019s syndrome (thymoma and hypogammaglobulinaemia)"],"prefix":"10.1093","volume":"195","author":[{"given":"M","family":"Zaman","sequence":"first","affiliation":[{"name":"Immunology, University of Manchester, Manchester University Hospitals NHS Trust, Manchester, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"A","family":"Huissoon","sequence":"additional","affiliation":[{"name":"West Midlands Immunodeficiency Centre, Birmingham Heartlands Hospital, Birmingham, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M","family":"Buckland","sequence":"additional","affiliation":[{"name":"Immunology, St Bartholomew\u2019s Hospital, London, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"S","family":"Patel","sequence":"additional","affiliation":[{"name":"Immunology, John Radcliffe Hospital, Oxford, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"H","family":"Alachkar","sequence":"additional","affiliation":[{"name":"Immunology, Salford Royal Foundation Trust, Manchester, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J D","family":"Edgar","sequence":"additional","affiliation":[{"name":"Regional Immunology Service, The Royal Hospitals, Belfast, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M","family":"Thomas","sequence":"additional","affiliation":[{"name":"Immunology, NHS Greater Glasgow and Clyde, Glasgow, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"G","family":"Arumugakani","sequence":"additional","affiliation":[{"name":"Immunology, Leeds Teaching Hospitals, Leeds, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"H","family":"Baxendale","sequence":"additional","affiliation":[{"name":"Immunology, Papworth Hospital, Cambridge, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"S","family":"Burns","sequence":"additional","affiliation":[{"name":"University College London, Immunology, Royal Free Hospital, London, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"A P","family":"Williams","sequence":"additional","affiliation":[{"name":"Immunology, Southampton General Hospital, Southampton, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"S","family":"Jolles","sequence":"additional","affiliation":[{"name":"Immunodeficiency Centre for Wales, University Hospital of Wales, Cardiff, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"R","family":"Herriot","sequence":"additional","affiliation":[{"name":"Immunology, Royal Aberdeen Infirmary, Aberdeen, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"R B","family":"Sargur","sequence":"additional","affiliation":[{"name":"Immunology, Northern General Hospital, Sheffield, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"P D","family":"Arkwright","sequence":"additional","affiliation":[{"name":"Immunology, University of Manchester, Manchester University Hospitals NHS Trust, Manchester, UK"},{"name":"Immunology, Papworth Hospital, Cambridge, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2018,10,21]]},"reference":[{"key":"2022011418453352500_cei13216-bib-0001","doi-asserted-by":"crossref","first-page":"244","DOI":"10.1016\/j.ccell.2018.01.003","article-title":"The integrated genomic landscape of thymic 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