Clinical & laboratory features of seventy-eight UK patients with Good's syndrome (thymoma & hypogammaglobulinemia).

Zaman, Maryiam and Huissoon, Aarnoud and Buckland, Matthew and Patel, Smita and Alachkar, Hana and Edgar, J David and Thomas, Moira and Arumugakani, Gururaj and Baxendale, Helen and Burns, Siobhan and Williams, Anthony P and Jolles, Stephen and Herriot, Richard and Sargur, Ravishankar B and Arkwright, Peter D (2018) Clinical & laboratory features of seventy-eight UK patients with Good's syndrome (thymoma & hypogammaglobulinemia). Clinical and experimental immunology. ISSN 1365-2249. This article is available to all HEFT staff and students via ASK Discovery tool http://tinyurl.com/z795c8c by using their HEFT Athens login IDs

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Official URL: https://doi.org/10.1111/cei.13216

Abstract

Good's syndrome (thymoma and hypogammaglobulinemia) 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 females 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 9 (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, SLE, Sjogren's syndrome). There was no association between thymoma type and autoimmunity. Seven (9%) patients had died. Good's 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 hypogammaglobulinemia. This article is protected by copyright. All rights reserved.

Item Type: Article
Additional Information: This article is available to all HEFT staff and students via ASK Discovery tool http://tinyurl.com/z795c8c by using their HEFT Athens login IDs
Subjects: QW Microbiology. Immunology
Divisions: Emergency Services > Acute Medicine and AMU
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Depositing User: Mr Philip O'Reilly
Date Deposited: 24 Sep 2018 14:03
Last Modified: 24 Sep 2018 14:03
URI: http://www.repository.heartofengland.nhs.uk/id/eprint/1747

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