Good’s syndrome, a rare form of acquired immunodeficiency associated with thymomas

  • Antonio Tamburello | antonio.tamburello@asst-ovestmi.it Department of Internal Medicine, Hospital of Legnano, Legnano (MI), Italy.
  • Laura Castelnovo Department of Internal Medicine, Hospital of Legnano, Legnano (MI), Italy.
  • Paola Faggioli Department of Internal Medicine, Hospital of Legnano, Legnano (MI), Italy.
  • Daniela Bompane Department of Internal Medicine, Hospital of Legnano, Legnano (MI), Italy.
  • Bruno Brando Hematology Laboratory and Transfusion Center, Hospital of Legnano, Legnano (MI), Italy.
  • Arianna Gatti Hematology Laboratory and Transfusion Center, Hospital of Legnano, Legnano (MI), Italy.
  • Lucia Roncoroni Department of Anatomical Pathology, Hospital of Legnano, Legnano (MI), Italy.
  • Biancamaria Di Marco Department of Anatomical Pathology, Hospital of Legnano, Legnano (MI), Italy.
  • Antonino Mazzone Department of Internal Medicine, Hospital of Legnano, Legnano (MI), Italy.

Abstract

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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Published
2019-06-05
Section
Case Reports
Keywords:
Immunodeficiency, Thymomas, Good’s syndrome, Hypogammaglobulinemia, Infections
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How to Cite
Tamburello, A., Castelnovo, L., Faggioli, P., Bompane, D., Brando, B., Gatti, A., Roncoroni, L., Di Marco, B., & Mazzone, A. (2019). Good’s syndrome, a rare form of acquired immunodeficiency associated with thymomas. Clinics and Practice, 9(2). https://doi.org/10.4081/cp.2019.1112