Good’s syndrome, a rare form of acquired immunodeficiency associated with thymomas
Good’s syndrome (GS) or thymomaassociated immunodeficiency is a rare clinical entity that should be ruled out in patients with thymoma who develop severe, recurrent bacterial infections and opportunistic viral and fungal infections. There are no treatment protocols established, hence, early recogni...
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doaj-6000b9422e6a4c86a1985bdcb4a47fda2021-04-02T16:50:53ZengMDPI AGClinics and Practice2039-72752039-72832019-06-019210.4081/cp.2019.1112Good’s syndrome, a rare form of acquired immunodeficiency associated with thymomasAntonio Tamburello0Laura Castelnovo1Paola Faggioli2Daniela Bompane3Bruno Brando4Arianna Gatti5Lucia Roncoroni6Biancamaria Di Marco7Antonino Mazzone8Department of Internal Medicine, Hospital of Legnano, Legnano (MI)Department of Internal Medicine, Hospital of Legnano, Legnano (MI)Department of Internal Medicine, Hospital of Legnano, Legnano (MI)Department of Internal Medicine, Hospital of Legnano, Legnano (MI)Hematology Laboratory and Transfusion Center, Hospital of Legnano, Legnano (MI)Hematology Laboratory and Transfusion Center, Hospital of Legnano, Legnano (MI)Department of Anatomical Pathology, Hospital of Legnano, Legnano (MI)Department of Anatomical Pathology, Hospital of Legnano, Legnano (MI)Department of Internal Medicine, Hospital of Legnano, Legnano (MI) Good’s syndrome (GS) or thymomaassociated immunodeficiency is a rare clinical entity that should be ruled out in patients with thymoma who develop severe, recurrent bacterial infections and opportunistic viral and fungal infections. There are no treatment protocols established, hence, early recognition is imperative to avoid complications. We report the case of a 42-year-old female, known for a previous thymectomy for giant thymoma who has suffered for a long time from recurrent pulmonary and urinary tract infections and cold sores. In March 2016 she referred to our unit complaining of fever, cough, chest pain, and cold sores due to Herpes simplex virus (HSV), confirmed serologically as HSV-1. Chest X-ray showed left pneumonia due to Streptococcus pneumoniae. She started antibiotics (amoxicillin/clavulanic acid associated with azithromycin) with gradual improvement. Given her history she was studied for an underlying immunodeficiency: IgG, IgA, and IgM were significantly low or absent, as well as all IgG subclasses; blood and bone marrow aspirate leucocyte immunophenotyping showed complete absence of B lymphocytes and reduced CD4+ T cells. In light of: i) thymoma; ii) B lymphocyte deficit; iii) hypogammaglobulinemia; iv) recurrent infections, GS was diagnosed and pre-emptive immunoglobulin treatment, associated with HSV and Pneumocystis jiroveci prophylaxis (Acyclovir for HSV and Sulfamethoxazole- Trimethoprim for P. jiroveci) were started. Since then the patient has no longer presented any infectious episodes. https://www.clinicsandpractice.org/index.php/cp/article/view/1112ImmunodeficiencyThymomasGood’s syndromeHypogammaglobulinemiaInfections |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Antonio Tamburello Laura Castelnovo Paola Faggioli Daniela Bompane Bruno Brando Arianna Gatti Lucia Roncoroni Biancamaria Di Marco Antonino Mazzone |
spellingShingle |
Antonio Tamburello Laura Castelnovo Paola Faggioli Daniela Bompane Bruno Brando Arianna Gatti Lucia Roncoroni Biancamaria Di Marco Antonino Mazzone Good’s syndrome, a rare form of acquired immunodeficiency associated with thymomas Clinics and Practice Immunodeficiency Thymomas Good’s syndrome Hypogammaglobulinemia Infections |
author_facet |
Antonio Tamburello Laura Castelnovo Paola Faggioli Daniela Bompane Bruno Brando Arianna Gatti Lucia Roncoroni Biancamaria Di Marco Antonino Mazzone |
author_sort |
Antonio Tamburello |
title |
Good’s syndrome, a rare form of acquired immunodeficiency associated with thymomas |
title_short |
Good’s syndrome, a rare form of acquired immunodeficiency associated with thymomas |
title_full |
Good’s syndrome, a rare form of acquired immunodeficiency associated with thymomas |
title_fullStr |
Good’s syndrome, a rare form of acquired immunodeficiency associated with thymomas |
title_full_unstemmed |
Good’s syndrome, a rare form of acquired immunodeficiency associated with thymomas |
title_sort |
good’s syndrome, a rare form of acquired immunodeficiency associated with thymomas |
publisher |
MDPI AG |
series |
Clinics and Practice |
issn |
2039-7275 2039-7283 |
publishDate |
2019-06-01 |
description |
Good’s syndrome (GS) or thymomaassociated immunodeficiency is a rare clinical entity that should be ruled out in patients with thymoma who develop severe, recurrent bacterial infections and opportunistic viral and fungal infections. There are no treatment protocols established, hence, early recognition is imperative to avoid complications. We report the case of a 42-year-old female, known for a previous thymectomy for giant thymoma who has suffered for a long time from recurrent pulmonary and urinary tract infections and cold sores. In March 2016 she referred to our unit complaining of fever, cough, chest pain, and cold sores due to Herpes simplex virus (HSV), confirmed serologically as HSV-1. Chest X-ray showed left pneumonia due to Streptococcus pneumoniae. She started antibiotics (amoxicillin/clavulanic acid associated with azithromycin) with gradual improvement. Given her history she was studied for an underlying immunodeficiency: IgG, IgA, and IgM were significantly low or absent, as well as all IgG subclasses; blood and bone marrow aspirate leucocyte immunophenotyping showed complete absence of B lymphocytes and reduced CD4+ T cells. In light of: i) thymoma; ii) B lymphocyte deficit; iii) hypogammaglobulinemia; iv) recurrent infections, GS was diagnosed and pre-emptive immunoglobulin treatment, associated with HSV and Pneumocystis jiroveci prophylaxis (Acyclovir for HSV and Sulfamethoxazole- Trimethoprim for P. jiroveci) were started. Since then the patient has no longer presented any infectious episodes.
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topic |
Immunodeficiency Thymomas Good’s syndrome Hypogammaglobulinemia Infections |
url |
https://www.clinicsandpractice.org/index.php/cp/article/view/1112 |
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