Clinical and laboratory features of seventy-eight UK patients with Good's syndrome (thymoma and hypogammaglobulinaemia)

Clinical and Experimental Immunology
M ZamanP D Arkwright

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
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
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
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

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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
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
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

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Methods Mentioned

BETA
flow cytometry

Software Mentioned

SPSS Statistics

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