Abstract
Good's 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 bronchie...Continue Reading
References
Jan 1, 1979·Journal of Clinical Pathology. Supplement (Royal College of Pathologists)·G L AshersonA D Webster
Mar 1, 1975·British Journal of Haematology·C G GearyJ Zervas
Sep 1, 1992·Surgical Neurology·E S NussbaumW L Robinson
Jan 1, 1991·The Annals of Thoracic Surgery·G MaggiE Ruffini
Jan 1, 1990·Acta otorhinolaryngologica Italica : organo ufficiale della Società italiana di otorinolaringologia e chirurgia cervico-facciale·G MaggiM Molinatti
Oct 1, 1996·British Journal of Haematology·D YipB O'Neill
Jan 8, 2003·Blood·Anna Maria MasciLuigi Racioppi
Jul 14, 2006·American Journal of Hematology·Giovannella PalmieriBruno Rotoli
Feb 13, 2010·Clinical Immunology : the Official Journal of the Clinical Immunology Society·Theodoros Kelesidis, Otto Yang
Mar 29, 2012·The Kaohsiung Journal of Medical Sciences·Ling-Ping ChenShyh-Jer Lin
Jul 7, 2012·Journal of Pediatric Hematology/oncology·Bilgehan YalçinMünevver Büyükpamukçu
Jul 1, 2014·Nature Genetics·Iacopo PetriniGiuseppe Giaccone
Nov 15, 2014·Journal of Thoracic Oncology : Official Publication of the International Association for the Study of Lung Cancer·Amelia Evoli, Eric Lancaster
Mar 3, 2015·The Journal of Allergy and Clinical Immunology·Consuelo AnzilottiHelen Chapel
Apr 2, 2015·Clinical Infectious Diseases : an Official Publication of the Infectious Diseases Society of America·Marion MalphettesUNKNOWN DEFicit Immunitaire de l'adulte Study Group
Aug 22, 2015·Journal of Thoracic Oncology : Official Publication of the International Association for the Study of Lung Cancer·Alexander MarxPhilipp Ströbel
Dec 4, 2015·Medicine·Xuefeng SunYi Xiao
Dec 10, 2015·Clinical and Experimental Immunology·J BrentP D Arkwright
Jan 4, 2017·Seminars in Thoracic and Cardiovascular Surgery·Andrew J KaufmanRaja M Flores
May 17, 2017·Chinese Journal of Cancer·Zu-Yang YuanJie He
Jun 24, 2017·Chinese Medical Journal·Jin-Pei DongHua-Hong Wang
Aug 31, 2017·Human Pathology·Annikka WeissferdtCesar A Moran
Feb 14, 2018·Cancer Cell·Milan RadovichPatrick J Loehrer
Citations
Apr 11, 2019·Therapeutic Advances in Neurological Disorders·Luisa KlotzHeinz Wiendl
Dec 14, 2019·ACG Case Reports Journal·Alejandro Campos-MurguíaSergio L Botello-Partida
Jan 12, 2021·Clinical Rheumatology·Yanxia ChenJinlin Liu
Jan 12, 2021·Modern Rheumatology·Yueting LiFengchun Zhang
Nov 15, 2020·Rheumatology·Marvyn T KoningA Faiz Karim
Nov 13, 2020·Asia Pacific Allergy·Yi Wye Lai, Teck-Choon Tan
Mar 7, 2021·Virchows Archiv : an International Journal of Pathology·Alexander MarxYosuke Yamada
Mar 16, 2021·Internal Medicine·Kyoko GochoTamiko Takemura
Apr 15, 2021·Open Medicine·Yi-Dan ChenYu-Fang Wang
May 11, 2021·Radiology. Cardiothoracic Imaging·Jose A RodriguezDaniel Vargas
Jun 12, 2021·Frontiers in Immunology·Yiyun Shi, Chen Wang
Jul 15, 2021·Seminars in Respiratory and Critical Care Medicine·Pamela J McShane
Aug 5, 2021·BMC Immunology·Jinyao NiJinlin Liu
Aug 14, 2021·Hematology·Weiwei ChenJinlin Liu