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.
Highlights
Thymoma is an uncommon and slowgrowing thymic epithelial, a rare malignancy that arises from the epithelium of the thymic gland
Good’s syndrome (GS) was first reported by Robert Good in 1954 and it is usually characterized by thymoma, hypogammaglobulinemia, low or absent Bcells, decreased T-cells, an inverted CD4+/CD8+ T-cell ratio and reduced T-cell mitogen proliferative responses.[2]
In March 2016, the patient referred to our Emergency Department complaining fever, persistent cough and chest pain that developed during a recent bronchitis and that had been treated with levofloxacin; a previous chest X-ray was negative
Summary
Thymoma is an uncommon and slowgrowing thymic epithelial, a rare malignancy that arises from the epithelium of the thymic gland. Parathymic syndromes include myasthenia gravis, pure red cell aplasia, connective tissue disorders and acquired hypogammaglobulinemia or Good’s syndrome (GS) This clinical entity should be ruled out in patients with thymoma who develop severe, recurrent opportunistic infections.[1] GS was first reported by Robert Good in 1954 and it is usually characterized by thymoma, hypogammaglobulinemia, low or absent Bcells, decreased T-cells, an inverted CD4+/CD8+ T-cell ratio and reduced T-cell mitogen proliferative responses.[2] GS has no well established therapeutic schedule and the diagnosis can be difficult; thymectomy and immunoglobulin replacement treatment have become the major management approaches. Immunoglobulin replacement has been reported to improve outcome by reducing the infection rate in patients with GS3,4 and associated hypogammaglobulinemia
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